{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T13:15:01Z","timestamp":1785762901363,"version":"3.56.0"},"reference-count":175,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,5,15]],"date-time":"2024-05-15T00:00:00Z","timestamp":1715731200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2024,5,15]],"date-time":"2024-05-15T00:00:00Z","timestamp":1715731200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP15J03921"],"award-info":[{"award-number":["JP15J03921"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP18K07368"],"award-info":[{"award-number":["JP18K07368"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP18KK0239"],"award-info":[{"award-number":["JP18KK0239"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP19K17002"],"award-info":[{"award-number":["JP19K17002"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP19K08002"],"award-info":[{"award-number":["JP19K08002"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP21H05278"],"award-info":[{"award-number":["JP21H05278"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP22K07500"],"award-info":[{"award-number":["JP22K07500"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP22K15736"],"award-info":[{"award-number":["JP22K15736"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP17K10083"],"award-info":[{"award-number":["JP17K10083"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP20H03567"],"award-info":[{"award-number":["JP20H03567"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP22K18388"],"award-info":[{"award-number":["JP22K18388"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP23H02882"],"award-info":[{"award-number":["JP23H02882"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP22K07500"],"award-info":[{"award-number":["JP22K07500"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP21K06417"],"award-info":[{"award-number":["JP21K06417"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP18K06506"],"award-info":[{"award-number":["JP18K06506"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP22H04923"],"award-info":[{"award-number":["JP22H04923"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP25305030"],"award-info":[{"award-number":["JP25305030"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP15K09364"],"award-info":[{"award-number":["JP15K09364"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP17H01689"],"award-info":[{"award-number":["JP17H01689"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP18K07514"],"award-info":[{"award-number":["JP18K07514"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP18K07368"],"award-info":[{"award-number":["JP18K07368"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP20H00485"],"award-info":[{"award-number":["JP20H00485"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP21F21410"],"award-info":[{"award-number":["JP21F21410"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP21H05273"],"award-info":[{"award-number":["JP21H05273"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP22KF0333"],"award-info":[{"award-number":["JP22KF0333"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP23bm1123046"],"award-info":[{"award-number":["JP23bm1123046"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP23kk0305024"],"award-info":[{"award-number":["JP23kk0305024"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP22bm0804023"],"award-info":[{"award-number":["JP22bm0804023"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP22gm6510006"],"award-info":[{"award-number":["JP22gm6510006"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP21wm0425019"],"award-info":[{"award-number":["JP21wm0425019"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP17ek0109139"],"award-info":[{"award-number":["JP17ek0109139"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP22bm0804003"],"award-info":[{"award-number":["JP22bm0804003"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP20ek0109395"],"award-info":[{"award-number":["JP20ek0109395"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP20ek0109329"],"award-info":[{"award-number":["JP20ek0109329"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP21wm0425009"],"award-info":[{"award-number":["JP21wm0425009"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP23bm1423002"],"award-info":[{"award-number":["JP23bm1423002"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100020050","name":"Japan Intractable Diseases Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100020050","id-type":"DOI","asserted-by":"publisher"}]},{"name":"The Kanae Foundation for the Promotion of Medical Science"},{"DOI":"10.13039\/100008732","name":"Uehara Memorial Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008732","id-type":"DOI","asserted-by":"publisher"}]},{"name":"THE YUKIHIKO MIYATA MEMORIAL TRUST FOR ALS RESEARCH"},{"name":"Okasan-Kato Foundation Research Grant"},{"name":"Yoshio Koide Grant, Japan ALS Association"},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Mie Medical Fund"},{"DOI":"10.13039\/100020235","name":"Japan Foundation for Neuroscience and Mental Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100020235","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Research Committee of CNS Degenerative Diseases, Research on Policy Planning and Evaluation for Rare and Intractable Diseases","award":["H29-Nanchi-Ippan-085"],"award-info":[{"award-number":["H29-Nanchi-Ippan-085"]}]},{"DOI":"10.13039\/100018254","name":"Japanese Foundation for Research and Promotion of Endoscopy","doi-asserted-by":"publisher","award":["JP18KK0239"],"award-info":[{"award-number":["JP18KK0239"]}],"id":[{"id":"10.13039\/100018254","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001700","name":"MEXT","doi-asserted-by":"crossref","award":["JP16H06277"],"award-info":[{"award-number":["JP16H06277"]}],"id":[{"id":"10.13039\/501100001700","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Intramural Research Grant for Neurological Psychiatric Disorders from NCNP"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Acta Neuropathol"],"published-print":{"date-parts":[[2024,6]]},"DOI":"10.1007\/s00401-024-02734-w","type":"journal-article","created":{"date-parts":[[2024,5,15]],"date-time":"2024-05-15T16:01:45Z","timestamp":1715788905000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Aberrant CHCHD2-associated\u00a0mitochondriopathy in Kii ALS\/PDC astrocytes"],"prefix":"10.1007","volume":"147","author":[{"given":"Nicolas","family":"Leventoux","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoru","family":"Morimoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitsuru","family":"Ishikawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shiho","family":"Nakamura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fumiko","family":"Ozawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reona","family":"Kobayashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirotaka","family":"Watanabe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sopak","family":"Supakul","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Okamoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhi","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroya","family":"Kobayashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chris","family":"Kato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshifumi","family":"Hirokawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ikuko","family":"Aiba","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinichi","family":"Takahashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinsuke","family":"Shibata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaki","family":"Takao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mari","family":"Yoshida","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fumito","family":"Endo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koji","family":"Yamanaka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasumasa","family":"Kokubo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7482-5935","authenticated-orcid":false,"given":"Hideyuki","family":"Okano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,5,15]]},"reference":[{"issue":"13","key":"2734_CR1","doi-asserted-by":"publisher","first-page":"e2107391119","DOI":"10.1073\/pnas.2107391119","volume":"119","author":"AA Almad","year":"2022","unstructured":"Almad AA, Taga A, Joseph J, Gross SK, Welsh C, Patankar A et al (2022) Cx43 hemichannels contribute to astrocyte-mediated toxicity in sporadic and familial ALS Proc Natl Acad Sci 119(13):e2107391119. https:\/\/doi.org\/10.1073\/pnas.2107391119","journal-title":"Cx43 hemichannels contribute to astrocyte-mediated toxicity in sporadic and familial ALS Proc Natl Acad Sci"},{"issue":"3","key":"2734_CR2","doi-asserted-by":"publisher","first-page":"602","DOI":"10.1016\/j.bbrc.2006.10.093","volume":"351","author":"T Arai","year":"2006","unstructured":"Arai T, Hasegawa M, Akiyama H, Ikeda K, Nonaka T, Mori H et al (2006) TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun 351(3):602\u2013611. https:\/\/doi.org\/10.1016\/j.bbrc.2006.10.093","journal-title":"Biochem Biophys Res Commun"},{"issue":"4","key":"2734_CR3","doi-asserted-by":"publisher","first-page":"2255","DOI":"10.1093\/nar\/gks1454","volume":"41","author":"S Aras","year":"2013","unstructured":"Aras S, Pak O, Sommer N, Finley R Jr, H\u00fcttemann M, Weissmann N et al (2013) Oxygen-dependent expression of cytochrome c oxidase subunit 4\u20132 gene expression is mediated by transcription factors RBPJ, CXXC5 and CHCHD2. Nucleic Acids Res 41(4):2255\u20132266. https:\/\/doi.org\/10.1093\/nar\/gks1454","journal-title":"Nucleic Acids Res"},{"key":"2734_CR4","doi-asserted-by":"publisher","first-page":"43","DOI":"10.1016\/j.mito.2014.10.003","volume":"20","author":"S Aras","year":"2015","unstructured":"Aras S, Bai M, Lee I, Springett R, H\u00fcttemann M, Grossman LI (2015) MNRR1 (formerly CHCHD2) is a bi-organellar regulator of mitochondrial metabolism. Mitochondrion 20:43\u201351. https:\/\/doi.org\/10.1016\/j.mito.2014.10.003","journal-title":"Mitochondrion"},{"issue":"8","key":"2734_CR5","doi-asserted-by":"publisher","first-page":"E736","DOI":"10.1073\/pnas.1222809110","volume":"110","author":"ES Arnold","year":"2013","unstructured":"Arnold ES, Ling SC, Huelga SC, Lagier-Tourenne C, Polymenidou M, Ditsworth D et al (2013) ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43. Proc Natl Acad Sci 110(8):E736\u2013E745. https:\/\/doi.org\/10.1073\/pnas.1222809110","journal-title":"Proc Natl Acad Sci"},{"issue":"1","key":"2734_CR6","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1038\/75556","volume":"25","author":"M Ashburner","year":"2000","unstructured":"Ashburner M, Ball CA, Blake JA, Botstein D, Butler H, Cherry JM et al (2000) Gene ontology: tool for the unification of biology. The Gene Ontology Consortium Nat Genet 25(1):25\u201329. https:\/\/doi.org\/10.1038\/75556","journal-title":"The Gene Ontology Consortium Nat Genet"},{"issue":"Pt 8","key":"2734_CR7","doi-asserted-by":"publisher","first-page":"2329","DOI":"10.1093\/brain\/awu138","volume":"137","author":"S Bannwarth","year":"2014","unstructured":"Bannwarth S, Ait-El-Mkadem S, Chaussenot A, Genin EC, Lacas-Gervais S, Fragaki K et al (2014) A mitochondrial origin for frontotemporal dementia and amyotrophic lateral sclerosis through CHCHD10 involvement. Brain 137(Pt 8):2329\u20132345. https:\/\/doi.org\/10.1093\/brain\/awu138","journal-title":"Brain"},{"issue":"2","key":"2734_CR8","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1016\/j.nbd.2006.06.017","volume":"24","author":"Z Batulan","year":"2006","unstructured":"Batulan Z, Taylor DM, Aarons RJ, Minotti S, Doroudchi MM, Nalbantoglu J et al (2006) Induction of multiple heat shock proteins and neuroprotection in a primary culture model of familial amyotrophic lateral sclerosis. Neurobiol Dis 24(2):213\u2013225. https:\/\/doi.org\/10.1016\/j.nbd.2006.06.017","journal-title":"Neurobiol Dis"},{"issue":"8","key":"2734_CR9","doi-asserted-by":"publisher","first-page":"e1000590","DOI":"10.1371\/journal.pgen.1000590Erratum.In:PLoSGenet6(3).10.1371\/annotation\/36fe7624-0904-46d4-a013-4be6195245c4","volume":"5","author":"JM Baughman","year":"2009","unstructured":"Baughman JM, Nilsson R, Gohil VM, Arlow DH, Gauhar Z, Mootha VK (2009) A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasis. PLoS Genet 5(8):e1000590. https:\/\/doi.org\/10.1371\/journal.pgen.1000590Erratum.In:PLoSGenet6(3).10.1371\/annotation\/36fe7624-0904-46d4-a013-4be6195245c4","journal-title":"PLoS Genet"},{"issue":"6","key":"2734_CR10","doi-asserted-by":"publisher","first-page":"689","DOI":"10.1007\/s00401-010-0664-3","volume":"119","author":"TG Beach","year":"2010","unstructured":"Beach TG, Adler CH, Sue LI, Vedders L, Lue L, White CL et al (2010) Arizona Parkinson\u2019s Disease Consortium. Multi organ distribution of phosphorylated alpha synuclein histopathology in subjects with Lewy body disorders. Acta Neuropathol 119(6):689\u2013702. https:\/\/doi.org\/10.1007\/s00401-010-0664-3","journal-title":"Acta Neuropathol"},{"issue":"4","key":"2734_CR11","first-page":"255","volume":"46","author":"E Bertrand","year":"2008","unstructured":"Bertrand E, Lewandowska E, Stepie\u0144 T, Szpak GM, Pasennik E, Modzelewska J (2008) Amyloid angiopathy in idiopathic Parkinson\u2019s disease. Immunohistochemical and ultrastructural study Folia Neuropathol 46(4):255\u2013270 (PMID: 19169967)","journal-title":"Immunohistochemical and ultrastructural study Folia Neuropathol"},{"issue":"9","key":"2734_CR12","doi-asserted-by":"publisher","first-page":"5062","DOI":"10.1093\/nar\/gkt189","volume":"41","author":"A Bhardwaj","year":"2013","unstructured":"Bhardwaj A, Myers MP, Buratti E, Baralle FE (2013) Characterizing TDP-43 interaction with its RNA targets. Nucleic Acids Res 41(9):5062\u20135074. https:\/\/doi.org\/10.1093\/nar\/gkt189","journal-title":"Nucleic Acids Res"},{"issue":"8","key":"2734_CR13","doi-asserted-by":"publisher","first-page":"1250","DOI":"10.1681\/ASN.2012121216","volume":"24","author":"AV Birk","year":"2013","unstructured":"Birk AV, Liu S, Soong Y, Mills W, Singh P, Warren JD et al (2013) The mitochondrial-targeted compound SS-31 re-energizes ischemic mitochondria by interacting with cardiolipin. J Am Soc Nephrol 24(8):1250\u20131261. https:\/\/doi.org\/10.1681\/ASN.2012121216","journal-title":"J Am Soc Nephrol"},{"issue":"6","key":"2734_CR14","doi-asserted-by":"publisher","first-page":"358","DOI":"10.1016\/j.tins.2017.04.001","volume":"40","author":"HDE Booth","year":"2017","unstructured":"Booth HDE, Hirst WD, Wade-Martins R (2017) The role of astrocyte dysfunction in Parkinson\u2019s disease pathogenesis. Trends Neurosci 40(6):358\u2013370. https:\/\/doi.org\/10.1016\/j.tins.2017.04.001","journal-title":"Trends Neurosci"},{"issue":"2","key":"2734_CR15","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1111\/jnc.14829","volume":"151","author":"MP Bordone","year":"2019","unstructured":"Bordone MP, Salman MM, Titus HE, Amini E, Andersen JV, Chakraborti B et al (2019) The energetic brain - a review from students to students. J Neurochem 151(2):139\u2013165. https:\/\/doi.org\/10.1111\/jnc.14829","journal-title":"J Neurochem"},{"issue":"6","key":"2734_CR16","doi-asserted-by":"publisher","first-page":"1687","DOI":"10.1111\/j.1460-9568.2006.05056.x","volume":"24","author":"AK Bouzier-Sore","year":"2006","unstructured":"Bouzier-Sore AK, Voisin P, Bouchaud V, Bezancon E, Franconi JM, Pellerin L (2006) Competition between glucose and lactate as oxidative energy substrates in both neurons and astrocytes: a comparative NMR study. Eur J Neurosci 24(6):1687\u20131694. https:\/\/doi.org\/10.1111\/j.1460-9568.2006.05056.x","journal-title":"Eur J Neurosci"},{"issue":"2","key":"2734_CR17","doi-asserted-by":"publisher","first-page":"342","DOI":"10.1006\/exnr.2001.7809","volume":"172","author":"DP Bradley","year":"2001","unstructured":"Bradley DP, Smith MI, Netsiri C, Smith JM, Bockhorst KH, Hall LD et al (2001) Diffusion-weighted MRI used to detect in vivo modulation of cortical spreading depression: comparison of sumatriptan and tonabersat. Exp Neurol 172(2):342\u2013353. https:\/\/doi.org\/10.1006\/exnr.2001.7809","journal-title":"Exp Neurol"},{"issue":"1","key":"2734_CR18","doi-asserted-by":"publisher","first-page":"264","DOI":"10.1523\/JNEUROSCI.4178-07.2008","volume":"28","author":"JD Cahoy","year":"2008","unstructured":"Cahoy JD, Emery B, Kaushal A, Foo LC, Zamanian JL, Christopherson KS et al (2008) A transcriptome database for astrocytes, neurons, and oligodendrocytes: a new resource for understanding brain development and function. J Neurosci 28(1):264\u2013278. https:\/\/doi.org\/10.1523\/JNEUROSCI.4178-07.2008","journal-title":"J Neurosci"},{"key":"2734_CR19","doi-asserted-by":"publisher","first-page":"104","DOI":"10.1016\/j.neurobiolaging.2017.08.023","volume":"60","author":"YL Cao","year":"2017","unstructured":"Cao YL, Yang YP, Mao CJ, Zhang XQ, Wang CT, Yang J et al (2017) A role of BAG3 in regulating SNCA\/\u03b1-synuclein clearance via selective macroautophagy. Neurobiol Aging 60:104\u2013115. https:\/\/doi.org\/10.1016\/j.neurobiolaging.2017.08.023","journal-title":"Neurobiol Aging"},{"issue":"3","key":"2734_CR20","doi-asserted-by":"publisher","first-page":"1437","DOI":"10.1074\/jbc.M706304200","volume":"283","author":"S Carra","year":"2008","unstructured":"Carra S, Seguin SJ, Lambert H, Landry J (2008) HspB8 chaperone activity toward poly(Q)-containing proteins depends on its association with Bag3, a stimulator of macroautophagy. J Biol Chem 283(3):1437\u20131444. https:\/\/doi.org\/10.1074\/jbc.M706304200","journal-title":"J Biol Chem"},{"issue":"17","key":"2734_CR21","doi-asserted-by":"publisher","first-page":"i884","DOI":"10.1093\/bioinformatics\/bty560","volume":"34","author":"S Chen","year":"2018","unstructured":"Chen S, Zhou Y, Chen Y, Gu J (2018) fastp: an ultra-fast all-in-one FASTQ preprocessor. Bioinformatics 34(17):i884\u2013i890. https:\/\/doi.org\/10.1093\/bioinformatics\/bty560","journal-title":"Bioinformatics"},{"issue":"1","key":"2734_CR22","doi-asserted-by":"publisher","first-page":"175","DOI":"10.1111\/j.1476-5381.2010.01041.x","volume":"162","author":"HJ Choi","year":"2011","unstructured":"Choi HJ, Kang KS, Fukui M, Zhu BT (2011) Critical role of the JNK-p53-GADD45\u03b1 apoptotic cascade in mediating oxidative cytotoxicity in hippocampal neurons. Br J Pharmacol 162(1):175\u2013192. https:\/\/doi.org\/10.1111\/j.1476-5381.2010.01041.x","journal-title":"Br J Pharmacol"},{"issue":"1","key":"2734_CR23","doi-asserted-by":"publisher","first-page":"160","DOI":"10.1016\/j.cell.2013.08.032","volume":"155","author":"S Cogliati","year":"2013","unstructured":"Cogliati S, Frezza C, Soriano ME, Varanita T, Quintana-Cabrera R, Corrado M et al (2013) Mitochondrial cristae shape determines respiratory chain supercomplexes assembly and respiratory efficiency. Cell 155(1):160\u2013171. https:\/\/doi.org\/10.1016\/j.cell.2013.08.032","journal-title":"Cell"},{"issue":"1\u20132","key":"2734_CR24","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1016\/s0169-328x(97)00125-3","volume":"49","author":"B Connor","year":"1997","unstructured":"Connor B, Young D, Yan Q, Faull RL, Synek B, Dragunow M (1997) Brain-derived neurotrophic factor is reduced in Alzheimer\u2019s disease. Brain Res Mol Brain Res 49(1\u20132):71\u201381. https:\/\/doi.org\/10.1016\/s0169-328x(97)00125-3","journal-title":"Brain Res Mol Brain Res"},{"key":"2734_CR25","first-page":"191","volume":"24","author":"D Dahl","year":"1981","unstructured":"Dahl D, Rueger DC, Bignami A, Weber K, Osborn M (1981) Vimentin, the 57,000 molecular weight protein of fibroblast filaments, is the major cytoskeletal component in immature glia. Eur J Cell Biol 24:191\u2013196 (PMID: 7285936)","journal-title":"Eur J Cell Biol"},{"issue":"1","key":"2734_CR26","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1016\/0006-8993(95)00063-v","volume":"676","author":"MC Dal Canto","year":"1995","unstructured":"Dal Canto MC, Gurney ME (1995) Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu, Zn SOD, and in mice overexpressing wild type human SOD: a model of familial amyotrophic lateral sclerosis (FALS). Brain Res 676(1):25\u201340. https:\/\/doi.org\/10.1016\/0006-8993(95)00063-v","journal-title":"Brain Res"},{"issue":"1","key":"2734_CR27","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/0306-4522(93)90175-f","volume":"52","author":"P Damier","year":"1993","unstructured":"Damier P, Hirsch EC, Zhang P, Agid Y, Javoy-Agid F (1993) Glutathione peroxidase, glial cells and Parkinson\u2019s disease. Neuroscience 52(1):1\u20136. https:\/\/doi.org\/10.1016\/0306-4522(93)90175-f","journal-title":"Neuroscience"},{"issue":"4","key":"2734_CR28","doi-asserted-by":"publisher","first-page":"2918","DOI":"10.1074\/jbc.M110.171975","volume":"286","author":"M Darshi","year":"2011","unstructured":"Darshi M, Mendiola VL, Mackey MR, Murphy AN, Koller A, Perkins GA et al (2011) ChChd3, an inner mitochondrial membrane protein, is essential for maintaining crista integrity and mitochondrial function. J Biol Chem 286(4):2918\u20132932. https:\/\/doi.org\/10.1074\/jbc.M110.171975","journal-title":"J Biol Chem"},{"key":"2734_CR29","doi-asserted-by":"publisher","first-page":"10148","DOI":"10.1073\/pnas.86.24.10148","volume":"86","author":"R Dermietzel","year":"1989","unstructured":"Dermietzel R, Traub O, Hwang TK, Beyer E, Bennett MV, Spray DC et al (1989) Differential expression of three gap junction proteins in developing and mature brain tissues. Proc Natl Acad Sci USA 86:10148\u201310152. https:\/\/doi.org\/10.1073\/pnas.86.24.10148","journal-title":"Proc Natl Acad Sci USA"},{"issue":"1","key":"2734_CR30","doi-asserted-by":"publisher","first-page":"949","DOI":"10.1152\/physrev.00062.2017","volume":"99","author":"GA Dienel","year":"2019","unstructured":"Dienel GA (2019) Brain Glucose Metabolism: Integration of Energetics with Function. Physiol Rev 99(1):949\u20131045. https:\/\/doi.org\/10.1152\/physrev.00062.2017","journal-title":"Physiol Rev"},{"issue":"5","key":"2734_CR31","doi-asserted-by":"publisher","first-page":"608","DOI":"10.1038\/nn1885","volume":"10","author":"FP Di Giorgio","year":"2007","unstructured":"Di Giorgio FP, Carrasco MA, Siao MC, Maniatis T, Eggan K (2007) Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model. Nat Neurosci 10(5):608\u2013614. https:\/\/doi.org\/10.1038\/nn1885","journal-title":"Nat Neurosci"},{"issue":"6","key":"2734_CR32","doi-asserted-by":"publisher","first-page":"637","DOI":"10.1016\/j.stem.2008.09.017","volume":"3","author":"FP Di Giorgio","year":"2008","unstructured":"Di Giorgio FP, Boulting GL, Bobrowicz S, Eggan KC (2008) Human embryonic stem cell-derived motor neurons are sensitive to the toxic effect of glial cells carrying an ALS-causing mutation. Cell Stem Cell 3(6):637\u2013648. https:\/\/doi.org\/10.1016\/j.stem.2008.09.017","journal-title":"Cell Stem Cell"},{"issue":"5","key":"2734_CR33","doi-asserted-by":"publisher","first-page":"16064","DOI":"10.1038\/srep16064","volume":"4","author":"C Ding","year":"2015","unstructured":"Ding C, Wu Z, Huang L, Wang Y, Xue J, Chen S et al (2015) Mitofilin and CHCHD6 physically interact with Sam50 to sustain cristae structure. Sci Rep 4(5):16064. https:\/\/doi.org\/10.1038\/srep16064","journal-title":"Sci Rep"},{"issue":"6","key":"2734_CR34","doi-asserted-by":"publisher","first-page":"1157","DOI":"10.1016\/s0896-6273(00)80247-0","volume":"17","author":"A Ebens","year":"1996","unstructured":"Ebens A, Brose K, Leonardo ED, Hanson MG Jr, Bladt F, Birchmeier C et al (1996) Hepatocyte growth factor\/scatter factor is an axonal chemoattractant and a neurotrophic factor for spinal motor neurons. Neuron 17(6):1157\u20131172. https:\/\/doi.org\/10.1016\/s0896-6273(00)80247-0","journal-title":"Neuron"},{"issue":"3","key":"2734_CR35","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1038\/s41593-020-00783-4","volume":"24","author":"C Escartin","year":"2021","unstructured":"Escartin C, Galea E, Lakatos A, O\u2019Callaghan JP, Petzold GC, Serrano-Pozo A et al (2021) Reactive astrocyte nomenclature, definitions, and future directions. Nat Neurosci 24(3):312\u2013325. https:\/\/doi.org\/10.1038\/s41593-020-00783-4","journal-title":"Nat Neurosci"},{"issue":"4","key":"2734_CR36","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.07739","volume":"28","author":"JR Friedman","year":"2015","unstructured":"Friedman JR, Mourier A, Yamada J, McCaffery JM, Nunnari J (2015) MICOS coordinates with respiratory complexes and lipids to establish mitochondrial inner membrane architecture. Elife 28(4):e07739. https:\/\/doi.org\/10.7554\/eLife.07739","journal-title":"Elife"},{"issue":"3","key":"2734_CR37","doi-asserted-by":"publisher","first-page":"274","DOI":"10.1016\/S1474-4422(14)70266-2","volume":"14","author":"M Funayama","year":"2015","unstructured":"Funayama M, Ohe K, Amo T, Furuya N, Yamaguchi J, Saiki S et al (2015) CHCHD2 mutations in autosomal dominant late-onset Parkinson\u2019s disease: a genome-wide linkage and sequencing study. Lancet Neurol 14(3):274\u2013282. https:\/\/doi.org\/10.1016\/S1474-4422(14)70266-2","journal-title":"Lancet Neurol"},{"issue":"21","key":"2734_CR38","doi-asserted-by":"publisher","first-page":"8363","DOI":"10.1523\/JNEUROSCI.17-21-08363.1997","volume":"17","author":"A Furuta","year":"1997","unstructured":"Furuta A, Rothstein JD, Martin LJ (1997) Glutamate transporter protein subtypes are expressed differentially during rat CNS development. J Neurosci 17(21):8363\u20138375. https:\/\/doi.org\/10.1523\/JNEUROSCI.17-21-08363.1997","journal-title":"J Neurosci"},{"issue":"7","key":"2734_CR39","doi-asserted-by":"publisher","first-page":"889","DOI":"10.1038\/emboj.2009.29","volume":"28","author":"M Gamerdinger","year":"2009","unstructured":"Gamerdinger M, Hajieva P, Kaya AM, Wolfrum U, Hartl FU, Behl C (2009) Protein quality control during aging involves recruitment of the macroautophagy pathway by BAG3. EMBO J 28(7):889\u2013901. https:\/\/doi.org\/10.1038\/emboj.2009.29","journal-title":"EMBO J"},{"issue":"1","key":"2734_CR40","doi-asserted-by":"publisher","first-page":"131","DOI":"10.1242\/dev.107.1.131","volume":"107","author":"SJ Gaunt","year":"1989","unstructured":"Gaunt SJ, Krumlauf R, Duboule D (1989) Mouse homeo-genes within a subfamily, Hox-1.4, -2.6 and -5.1, display similar anteroposterior domains of expression in the embryo, but show stage- and tissue-dependent differences in their regulation. Development 107(1):131\u2013141. https:\/\/doi.org\/10.1242\/dev.107.1.131","journal-title":"Development"},{"issue":"1","key":"2734_CR41","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1016\/j.ydbio.2015.10.033","volume":"409","author":"M Gazea","year":"2016","unstructured":"Gazea M, Tasouri E, Tolve M, Bosch V, Kabanova A, Gojak C et al (2016) Primary cilia are critical for Sonic hedgehog-mediated dopaminergic neurogenesis in the embryonic midbrain. Dev Biol 409(1):55\u201371. https:\/\/doi.org\/10.1016\/j.ydbio.2015.10.033","journal-title":"Dev Biol"},{"issue":"D1","key":"2734_CR42","doi-asserted-by":"publisher","first-page":"D325","DOI":"10.1093\/nar\/gkaa1113","volume":"49","author":"Gene Ontology Consortium","year":"2021","unstructured":"Gene Ontology Consortium (2021) The Gene Ontology resource: enriching a GOld mine. Nucleic Acids Res 49(D1):D325\u2013D334. https:\/\/doi.org\/10.1093\/nar\/gkaa1113","journal-title":"Nucleic Acids Res"},{"issue":"1","key":"2734_CR43","doi-asserted-by":"publisher","first-page":"58","DOI":"10.15252\/emmm.201505496","volume":"8","author":"EC Genin","year":"2016","unstructured":"Genin EC, Plutino M, Bannwarth S, Villa E, Cisneros-Barroso E, Roy M et al (2016) CHCHD10 mutations promote loss of mitochondrial cristae junctions with impaired mitochondrial genome maintenance and inhibition of apoptosis. EMBO Mol Med 8(1):58\u201372. https:\/\/doi.org\/10.15252\/emmm.201505496","journal-title":"EMBO Mol Med"},{"issue":"1","key":"2734_CR44","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1002\/path.5351","volume":"250","author":"JM Gregory","year":"2020","unstructured":"Gregory JM, Livesey MR, McDade K, Selvaraj BT, Barton SK, Chandran S et al (2020) Dysregulation of AMPA receptor subunit expression in sporadic ALS post-mortem brain. J Pathol 250(1):67\u201378. https:\/\/doi.org\/10.1002\/path.5351","journal-title":"J Pathol"},{"key":"2734_CR45","doi-asserted-by":"publisher","first-page":"6739236","DOI":"10.1155\/2017\/6739236","volume":"2017","author":"LI Grossman","year":"2017","unstructured":"Grossman LI, Purandare N, Arshad R, Gladyck S, Somayajulu M, H\u00fcttemann M et al (2017) MNRR1, a biorganellar regulator of mitochondria. Oxid Med Cell Longev 2017:6739236. https:\/\/doi.org\/10.1155\/2017\/6739236","journal-title":"Oxid Med Cell Longev"},{"issue":"1","key":"2734_CR46","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1002\/ana.21425","volume":"64","author":"M Hasegawa","year":"2008","unstructured":"Hasegawa M, Arai T, Nonaka T, Kametani F, Yoshida M, Hashizume Y et al (2008) Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Ann Neurol 64(1):60\u201370. https:\/\/doi.org\/10.1002\/ana.21425","journal-title":"Ann Neurol"},{"issue":"11","key":"2734_CR47","doi-asserted-by":"publisher","first-page":"751","DOI":"10.3389\/fnins.2017.00751","volume":"22","author":"Y Hata","year":"2018","unstructured":"Hata Y, Ma N, Yoneda M, Morimoto S, Okano H, Murayama S et al (2018) Nitrative stress and tau accumulation in amyotrophic lateral sclerosis\/parkinsonism-dementia complex (ALS\/PDC) in the Kii Peninsula. Japan Front Neurosci 22(11):751. https:\/\/doi.org\/10.3389\/fnins.2017.00751","journal-title":"Japan Front Neurosci"},{"issue":"8","key":"2734_CR48","doi-asserted-by":"publisher","first-page":"718","DOI":"10.1016\/S1474-4422(09)70135-8","volume":"8","author":"AW Hauge","year":"2009","unstructured":"Hauge AW, Asghar MS, Schytz HW, Christensen K, Olesen J (2009) Effects of tonabersat on migraine with aura: a randomised, double-blind, placebo-controlled crossover study. Lancet Neurol 8(8):718\u2013723. https:\/\/doi.org\/10.1016\/S1474-4422(09)70135-8","journal-title":"Lancet Neurol"},{"issue":"6","key":"2734_CR49","doi-asserted-by":"publisher","first-page":"582","DOI":"10.1111\/neup.12223","volume":"35","author":"S Hino","year":"2015","unstructured":"Hino S, Sasaki S (2015) Flail arm syndrome with cytoplasmic vacuoles in remaining anterior horn motor neurons: a peculiar variant of amyotrophic lateral sclerosis. Neuropathology 35(6):582\u2013586. https:\/\/doi.org\/10.1111\/neup.12223","journal-title":"Neuropathology"},{"issue":"1","key":"2734_CR50","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1006\/exnr.2000.7483","volume":"166","author":"DW Howells","year":"2000","unstructured":"Howells DW, Porritt MJ, Wong JY, Batchelor PE, Kalnins R, Hughes AJ et al (2000) Reduced BDNF mRNA expression in the Parkinson\u2019s disease substantia nigra. Exp Neurol 166(1):127\u2013135. https:\/\/doi.org\/10.1006\/exnr.2000.7483","journal-title":"Exp Neurol"},{"key":"2734_CR51","doi-asserted-by":"publisher","first-page":"3227","DOI":"10.1038\/ncomms4227","volume":"5","author":"EM Hwang","year":"2014","unstructured":"Hwang EM, Kim E, Yarishkin O, Woo DH, Han KS, Park N et al (2014) A disulphide-linked heterodimer of TWIK-1 and TREK-1 mediates passive conductance in astrocytes. Nat Commun 5:3227. https:\/\/doi.org\/10.1038\/ncomms4227","journal-title":"Nat Commun"},{"issue":"1","key":"2734_CR52","doi-asserted-by":"publisher","first-page":"e202203019","DOI":"10.1083\/jcb.202203019","volume":"222","author":"O Ignatenko","year":"2023","unstructured":"Ignatenko O, Malinen S, Rybas S, Vihinen H, Nikkanen J, Kononov A et al (2023) Mitochondrial dysfunction compromises ciliary homeostasis in astrocytes. J Cell Biol 222(1):e202203019. https:\/\/doi.org\/10.1083\/jcb.202203019","journal-title":"J Cell Biol"},{"issue":"9","key":"2734_CR53","doi-asserted-by":"publisher","first-page":"e202000797","DOI":"10.26508\/lsa.202000797","volume":"3","author":"O Ignatenko","year":"2020","unstructured":"Ignatenko O, Nikkanen J, Kononov A, Zamboni N, Ince-Dunn G, Suomalainen A (2020) Mitochondrial spongiotic brain disease: astrocytic stress and harmful rapamycin and ketosis effect. Life Sci Alliance 3(9):e202000797. https:\/\/doi.org\/10.26508\/lsa.202000797","journal-title":"Life Sci Alliance"},{"issue":"23","key":"2734_CR54","doi-asserted-by":"publisher","first-page":"3895","DOI":"10.1093\/hmg\/ddz241","volume":"28","author":"A Ikeda","year":"2019","unstructured":"Ikeda A, Nishioka K, Meng H, Takanashi M, Hasegawa I, Inoshita T et al (2019) Mutations in CHCHD2 cause \u03b1-synuclein aggregation. Hum Mol Genet 28(23):3895\u20133911. https:\/\/doi.org\/10.1093\/hmg\/ddz241","journal-title":"Hum Mol Genet"},{"issue":"3","key":"2734_CR55","doi-asserted-by":"publisher","first-page":"532","DOI":"10.3390\/cells9030532","volume":"9","author":"M Ishikawa","year":"2020","unstructured":"Ishikawa M, Aoyama T, Shibata S, Sone T, Miyoshi H, Watanabe H et al (2020) miRNA-Based Rapid Differentiation of Purified Neurons from hPSCs Advances towards Quick Screening for Neuronal Disease Phenotypes In Vitro. Cells 9(3):532. https:\/\/doi.org\/10.3390\/cells9030532","journal-title":"Cells"},{"issue":"1","key":"2734_CR56","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1093\/brain\/awac306","volume":"146","author":"D Ito","year":"2023","unstructured":"Ito D, Morimoto S, Takahashi S, Okada K, Nakahara J, Okano H (2023) Maiden voyage: induced pluripotent stem cell-based drug screening for amyotrophic lateral sclerosis. Brain 146(1):13\u201319. https:\/\/doi.org\/10.1093\/brain\/awac306","journal-title":"Brain"},{"issue":"2","key":"2734_CR57","doi-asserted-by":"publisher","first-page":"313","DOI":"10.1006\/bbrc.1997.6943","volume":"236","author":"K Itoh","year":"1997","unstructured":"Itoh K, Chiba T, Takahashi S, Ishii T, Igarashi K, Katoh Y et al (1997) An Nrf2\/small Maf heterodimer mediates the induction of phase II detoxifying enzyme genes through antioxidant response elements. Biochem Biophys Res Commun 236(2):313\u2013322. https:\/\/doi.org\/10.1006\/bbrc.1997.6943","journal-title":"Biochem Biophys Res Commun"},{"issue":"7","key":"2734_CR58","doi-asserted-by":"publisher","first-page":"678","DOI":"10.1016\/S1474-4422(15)00094-0","volume":"14","author":"IE Jansen","year":"2015","unstructured":"Jansen IE, Bras JM, Lesage S, Schulte C, Gibbs JR, Nalls MA et al (2015) CHCHD2 and Parkinson\u2019s disease. Lancet Neurol 14(7):678\u2013679. https:\/\/doi.org\/10.1016\/S1474-4422(15)00094-0","journal-title":"Lancet Neurol"},{"issue":"1","key":"2734_CR59","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1093\/nar\/28.1.27","volume":"28","author":"M Kanehisa","year":"2000","unstructured":"Kanehisa M, Goto S (2000) KEGG: kyoto encyclopedia of genes and genomes. Nucleic Acids Res 28(1):27\u201330. https:\/\/doi.org\/10.1093\/nar\/28.1.27","journal-title":"Nucleic Acids Res"},{"issue":"11","key":"2734_CR60","doi-asserted-by":"publisher","first-page":"1947","DOI":"10.1002\/pro.3715","volume":"28","author":"M Kanehisa","year":"2019","unstructured":"Kanehisa M (2019) Toward understanding the origin and evolution of cellular organisms. Protein Sci 28(11):1947\u20131951. https:\/\/doi.org\/10.1002\/pro.3715","journal-title":"Protein Sci"},{"issue":"D1","key":"2734_CR61","doi-asserted-by":"publisher","first-page":"D587","DOI":"10.1093\/nar\/gkac963","volume":"51","author":"M Kanehisa","year":"2023","unstructured":"Kanehisa M, Furumichi M, Sato Y, Kawashima M, Ishiguro-Watanabe M (2023) KEGG for taxonomy-based analysis of pathways and genomes. Nucleic Acids Res 51(D1):D587\u2013D592. https:\/\/doi.org\/10.1093\/nar\/gkac963","journal-title":"Nucleic Acids Res"},{"issue":"4","key":"2734_CR62","doi-asserted-by":"publisher","first-page":"402","DOI":"10.1038\/nm1021","volume":"10","author":"D Kieran","year":"2004","unstructured":"Kieran D, Kalmar B, Dick JR, Riddoch-Contreras J, Burnstock G, Greensmith L (2004) Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice. Nat Med 10(4):402\u2013405. https:\/\/doi.org\/10.1038\/nm1021","journal-title":"Nat Med"},{"issue":"1","key":"2734_CR63","doi-asserted-by":"publisher","first-page":"36","DOI":"10.1007\/s12011-014-0202-6","volume":"164","author":"T Kihira","year":"2015","unstructured":"Kihira T, Sakurai I, Yoshida S, Wakayama I, Takamiya K, Okumura R et al (2015) Neutron activation analysis of scalp hair from ALS patients and residents in the Kii Peninsula. Japan Biol Trace Elem Res 164(1):36\u201342. https:\/\/doi.org\/10.1007\/s12011-014-0202-6","journal-title":"Japan Biol Trace Elem Res"},{"issue":"4","key":"2734_CR64","doi-asserted-by":"publisher","first-page":"1148","DOI":"10.1007\/s13311-017-0536-9","volume":"14","author":"Y Kim","year":"2017","unstructured":"Kim Y, Griffin JM, Nor MNM, Zhang J, Freestone PS, Danesh-Meyer HV et al (2017) Tonabersat prevents inflammatory damage in the central nervous system by blocking connexin43 hemichannels. Neurotherapeutics 14(4):1148\u20131165. https:\/\/doi.org\/10.1007\/s13311-017-0536-9","journal-title":"Neurotherapeutics"},{"issue":"1","key":"2734_CR65","doi-asserted-by":"publisher","first-page":"5750","DOI":"10.1038\/s41598-018-24075-x","volume":"8","author":"S Kleinsimon","year":"2018","unstructured":"Kleinsimon S, Longmuss E, Rolff J, J\u00e4ger S, Eggert A, Delebinski C et al (2018) GADD45A and CDKN1A are involved in apoptosis and cell cycle modulatory effects of viscumTT with further inactivation of the STAT3 pathway. Sci Rep 8(1):5750. https:\/\/doi.org\/10.1038\/s41598-018-24075-x","journal-title":"Sci Rep"},{"issue":"12","key":"2734_CR66","doi-asserted-by":"publisher","first-page":"4511","DOI":"10.1007\/s10072-023-07000-7","volume":"44","author":"H Kobayashi","year":"2023","unstructured":"Kobayashi H, Ueda K, Morimoto S, Ishikawa M, Leventoux N, Sasaki R et al (2023) Protein profiling of extracellular vesicles from iPSC-derived astrocytes of patients with ALS\/PDC in Kii peninsula. Neurol Sci 44(12):4511\u20134516. https:\/\/doi.org\/10.1007\/s10072-023-07000-7","journal-title":"Neurol Sci"},{"issue":"12","key":"2734_CR67","doi-asserted-by":"publisher","first-page":"2399","DOI":"10.1212\/01.wnl.0000147241.52694.6a","volume":"63","author":"Y Kokubo","year":"2004","unstructured":"Kokubo Y, Kuzuhara S (2004) Neurofibrillary tangles in ALS and Parkinsonism-dementia complex focus in Kii. Japan. Neurology 63(12):2399\u20132401. https:\/\/doi.org\/10.1212\/01.wnl.0000147241.52694.6a","journal-title":"Japan. Neurology"},{"issue":"10","key":"2734_CR68","doi-asserted-by":"publisher","first-page":"1091","DOI":"10.1212\/WNL.0000000000004310","volume":"89","author":"Y Kokubo","year":"2017","unstructured":"Kokubo Y, Banack SA, Morimoto S, Murayama S, Togashi T, Metcalf JS et al (2017) \u03b2-N-methylamino-l-alanine analysis in the brains of patients with Kii ALS\/PDC. Neurology 89(10):1091\u20131092. https:\/\/doi.org\/10.1212\/WNL.0000000000004310","journal-title":"Neurology"},{"issue":"9","key":"2734_CR69","doi-asserted-by":"publisher","first-page":"1257","DOI":"10.1001\/archneur.60.9.1257","volume":"60","author":"Y Kokubo","year":"2003","unstructured":"Kokubo Y, Kuzuhara S (2003) Neuroradiological study of patients with amyotrophic lateral sclerosis and parkinsonism-dementia complex on the Kii peninsula of Japan. Arch Neurol 60(9):1257\u20131261. https:\/\/doi.org\/10.1001\/archneur.60.9.1257","journal-title":"Arch Neurol"},{"issue":"7","key":"2734_CR70","doi-asserted-by":"publisher","first-page":"625","DOI":"10.1097\/NEN.0b013e31825b9680","volume":"71","author":"Y Kokubo","year":"2012","unstructured":"Kokubo Y, Taniguchi A, Hasegawa M, Hayakawa Y, Morimoto S, Yoneda M et al (2012) \u03b1-Synuclein pathology in the amyotrophic lateral sclerosis\/parkinsonism dementia complex in the Kii Peninsula. Japan J Neuropathol Exp Neurol 71(7):625\u2013630. https:\/\/doi.org\/10.1097\/NEN.0b013e31825b9680","journal-title":"Japan J Neuropathol Exp Neurol"},{"issue":"6","key":"2734_CR71","doi-asserted-by":"publisher","first-page":"577","DOI":"10.1212\/WNL.0000000000002361","volume":"86","author":"E Koschmidder","year":"2016","unstructured":"Koschmidder E, Weissbach A, Br\u00fcggemann N, Kasten M, Klein C, Lohmann K (2016) A nonsense mutation in CHCHD2 in a patient with Parkinson disease. Neurology 86(6):577\u2013579. https:\/\/doi.org\/10.1212\/WNL.0000000000002361","journal-title":"Neurology"},{"issue":"12","key":"2734_CR72","doi-asserted-by":"publisher","first-page":"815","DOI":"10.5692\/clinicalneurol.cn-001688","volume":"61","author":"S Kuzuhara","year":"2021","unstructured":"Kuzuhara S (2021) \u201cEndemic paraplegia of Koza in Kii\u201d in Honcho Koji Innen Shu published in 1689 is probably the earliest description of amyotrophic lateral sclerosis of Kii Peninsula: Presentation of the original and investigation of factuality. Rinsho Shinkeigaku 61(12):815\u2013824. https:\/\/doi.org\/10.5692\/clinicalneurol.cn-001688","journal-title":"Rinsho Shinkeigaku"},{"issue":"4","key":"2734_CR73","doi-asserted-by":"publisher","first-page":"501","DOI":"10.1002\/ana.100","volume":"49","author":"S Kuzuhara","year":"2001","unstructured":"Kuzuhara S, Kokubo Y, Sasaki R, Narita Y, Yabana T, Hasegawa M et al (2001) Familial amyotrophic lateral sclerosis and parkinsonism-dementia complex of the Kii Peninsula of Japan: clinical and neuropathological study and tau analysis. Ann Neurol 49(4):501\u2013511. https:\/\/doi.org\/10.1002\/ana.100","journal-title":"Ann Neurol"},{"issue":"Suppl 12","key":"2734_CR74","doi-asserted-by":"publisher","first-page":"S108","DOI":"10.1002\/mds.20548","volume":"20","author":"S Kuzuhara","year":"2005","unstructured":"Kuzuhara S, Kokubo Y (2005) Atypical parkinsonism of Japan: amyotrophic lateral sclerosis-parkinsonism-dementia complex of the Kii peninsula of Japan (Muro disease): an update. Mov Disord 20(Suppl 12):S108\u2013S113. https:\/\/doi.org\/10.1002\/mds.20548","journal-title":"Mov Disord"},{"issue":"4","key":"2734_CR75","doi-asserted-by":"publisher","first-page":"630","DOI":"10.1016\/j.cmet.2019.09.001","volume":"30","author":"S Lautrup","year":"2019","unstructured":"Lautrup S, Sinclair DA, Mattson MP, Fang EF (2019) NAD+ Cell Metab 30(4):630\u2013655. https:\/\/doi.org\/10.1016\/j.cmet.2019.09.001","journal-title":"NAD+ Cell Metab"},{"issue":"7","key":"2734_CR76","doi-asserted-by":"publisher","first-page":"1297","DOI":"10.1016\/j.cellsig.2015.03.016","volume":"27","author":"S Lecat","year":"2015","unstructured":"Lecat S, Belemnaba L, Galzi JL, Bucher B (2015) Neuropeptide Y receptor mediates activation of ERK1\/2 via transactivation of the IGF receptor. Cell Signal 27(7):1297\u20131304. https:\/\/doi.org\/10.1016\/j.cellsig.2015.03.016","journal-title":"Cell Signal"},{"key":"2734_CR77","doi-asserted-by":"publisher","first-page":"101896","DOI":"10.1016\/j.scr.2020.101896","volume":"47","author":"N Leventoux","year":"2020","unstructured":"Leventoux N, Morimoto S, Hara K, Nakamura S, Ozawa F, Mitsuzawa S et al (2020) Generation of an ALS human iPSC line KEIOi001 A from peripheral blood of a Charcot disease-affected patient carrying TARDBP p. N345K heterozygous SNP mutation. Stem Cell Res 47:101896. https:\/\/doi.org\/10.1016\/j.scr.2020.101896","journal-title":"Stem Cell Res"},{"issue":"12","key":"2734_CR78","doi-asserted-by":"publisher","first-page":"2680","DOI":"10.3390\/cells9122680","volume":"9","author":"N Leventoux","year":"2020","unstructured":"Leventoux N, Morimoto S, Imaizumi K, Sato Y, Takahashi S, Mashima K et al (2020) Human Astrocytes Model Derived from Induced Pluripotent Stem Cells. Cells 9(12):2680. https:\/\/doi.org\/10.3390\/cells9122680","journal-title":"Cells"},{"issue":"7638","key":"2734_CR79","doi-asserted-by":"publisher","first-page":"481","DOI":"10.1038\/nature21029","volume":"541","author":"SA Liddelow","year":"2017","unstructured":"Liddelow SA, Guttenplan KA, Clarke LE, Bennett FC, Bohlen CJ, Schirmer L et al (2017) Neurotoxic reactive astrocytes are induced by activated microglia. Nature 541(7638):481\u2013487. https:\/\/doi.org\/10.1038\/nature21029","journal-title":"Nature"},{"issue":"1","key":"2734_CR80","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1016\/j.ydbio.2004.08.018","volume":"276","author":"Y Liu","year":"2004","unstructured":"Liu Y, Han SS, Wu Y, Tuohy TM, Xue H, Cai J et al (2004) CD44 expression identifies astrocyte-restricted precursor cells. Dev Biol 276(1):31\u201346. https:\/\/doi.org\/10.1016\/j.ydbio.2004.08.018","journal-title":"Dev Biol"},{"issue":"4","key":"2734_CR81","doi-asserted-by":"publisher","first-page":"e1004964","DOI":"10.1080\/23723556.2015.1004964","volume":"2","author":"Y Liu","year":"2015","unstructured":"Liu Y, Zhang Y (2015) CHCHD2 connects mitochondrial metabolism to apoptosis. Mol Cell Oncol 2(4):e1004964. https:\/\/doi.org\/10.1080\/23723556.2015.1004964","journal-title":"Mol Cell Oncol"},{"key":"2734_CR82","doi-asserted-by":"publisher","DOI":"10.1016\/j.exer.2020.108384","volume":"202","author":"HH Louie","year":"2021","unstructured":"Louie HH, Shome A, Kuo CY, Rupenthal ID, Green CR, Mugisho OO (2021) Connexin43 hemichannel block inhibits NLRP3 inflammasome activation in a human retinal explant model of diabetic retinopathy. Exp Eye Res 202:108384. https:\/\/doi.org\/10.1016\/j.exer.2020.108384","journal-title":"Exp Eye Res"},{"issue":"12","key":"2734_CR83","doi-asserted-by":"publisher","first-page":"550","DOI":"10.1186\/s13059-014-0550-8","volume":"15","author":"MI Love","year":"2014","unstructured":"Love MI, Huber W, Anders S (2014) Moderated estimation of fold change and dispersion for RNA-seq data with DESeq2. Genome Biol 15(12):550. https:\/\/doi.org\/10.1186\/s13059-014-0550-8","journal-title":"Genome Biol"},{"issue":"3\u20134","key":"2734_CR84","doi-asserted-by":"publisher","first-page":"353","DOI":"10.1016\/j.biochi.2004.10.006","volume":"87","author":"FX Maquart","year":"2005","unstructured":"Maquart FX, Bellon G, Pasco S, Monboisse JC (2005) Matrikines in the regulation of extracellular matrix degradation. Biochimie 87(3\u20134):353\u2013360. https:\/\/doi.org\/10.1016\/j.biochi.2004.10.006","journal-title":"Biochimie"},{"issue":"6","key":"2734_CR85","doi-asserted-by":"publisher","first-page":"649","DOI":"10.1016\/j.stem.2008.10.001","volume":"3","author":"MC Marchetto","year":"2008","unstructured":"Marchetto MC, Muotri AR, Mu Y, Smith AM, Cezar GG, Gage FH (2008) Non-cell-autonomous effect of human SOD1 G37R astrocytes on motor neurons derived from human embryonic stem cells. Cell Stem Cell 3(6):649\u2013657. https:\/\/doi.org\/10.1016\/j.stem.2008.10.001","journal-title":"Cell Stem Cell"},{"issue":"3","key":"2734_CR86","doi-asserted-by":"publisher","first-page":"422","DOI":"10.1016\/j.stemcr.2016.01.010","volume":"6","author":"T Matsumoto","year":"2016","unstructured":"Matsumoto T, Fujimori K, Andoh-Noda T, Ando T, Kuzumaki N, Toyoshima M et al (2016) Functional Neurons Generated from T Cell-Derived Induced Pluripotent Stem Cells for Neurological Disease Modeling. Stem Cell Reports 6(3):422\u2013435. https:\/\/doi.org\/10.1016\/j.stemcr.2016.01.010","journal-title":"Stem Cell Reports"},{"issue":"6","key":"2734_CR87","doi-asserted-by":"publisher","first-page":"670","DOI":"10.1007\/s00018-004-4464-6","volume":"62","author":"MP Mayer","year":"2005","unstructured":"Mayer MP, Bukau B (2005) Hsp70 chaperones: cellular functions and molecular mechanism. Cell Mol Life Sci 62(6):670\u2013684. https:\/\/doi.org\/10.1007\/s00018-004-4464-6","journal-title":"Cell Mol Life Sci"},{"issue":"4","key":"2734_CR88","doi-asserted-by":"publisher","first-page":"846","DOI":"10.1046\/j.1471-4159.2002.01190.x","volume":"83","author":"PJ McLean","year":"2002","unstructured":"McLean PJ, Kawamata H, Shariff S, Hewett J, Sharma N, Ueda K et al (2002) TorsinA and heat shock proteins act as molecular chaperones: suppression of alpha-synuclein aggregation. J Neurochem 83(4):846\u2013854. https:\/\/doi.org\/10.1046\/j.1471-4159.2002.01190.x","journal-title":"J Neurochem"},{"issue":"1446","key":"2734_CR89","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1016\/j.brainres.2011.12.064","volume":"29","author":"T Mimoto","year":"2012","unstructured":"Mimoto T, Miyazaki K, Morimoto N, Kurata T, Satoh K, Ikeda Y et al (2012) Impaired antioxydative Keap1\/Nrf2 system and the downstream stress protein responses in the motor neuron of ALS model mice. Brain Res 29(1446):109\u2013118. https:\/\/doi.org\/10.1016\/j.brainres.2011.12.064","journal-title":"Brain Res"},{"issue":"1","key":"2734_CR90","doi-asserted-by":"publisher","first-page":"98","DOI":"10.1111\/neup.12434","volume":"38","author":"M Mimuro","year":"2018","unstructured":"Mimuro M, Yoshida M, Kuzuhara S, Kokubo Y (2018) Amyotrophic lateral sclerosis and parkinsonism-dementia complex of the Hohara focus of the Kii Peninsula: a multiple proteinopathy? Neuropathology 38(1):98\u2013107. https:\/\/doi.org\/10.1111\/neup.12434","journal-title":"Neuropathology"},{"issue":"3","key":"2734_CR91","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1016\/j.tibs.2015.12.004","volume":"41","author":"N Modjtahedi","year":"2016","unstructured":"Modjtahedi N, Tokatlidis K, Dessen P, Kroemer G (2016) Mitochondrial proteins containing coiled-coil-helix-coiled-coil-helix (CHCH) domains in health and disease. Trends Biochem Sci 41(3):245\u2013260. https:\/\/doi.org\/10.1016\/j.tibs.2015.12.004","journal-title":"Trends Biochem Sci"},{"issue":"16","key":"2734_CR92","doi-asserted-by":"publisher","first-page":"5724","DOI":"10.3390\/ijms21165724","volume":"21","author":"CA Morato Torres","year":"2020","unstructured":"Morato Torres CA, Wassouf Z, Zafar F, Sastre D, Outeiro TF, Sch\u00fcle B (2020) The role of alpha-synuclein and other Parkinson\u2019s genes in neurodevelopmental and neurodegenerative disorders. Int J Mol Sci 21(16):5724. https:\/\/doi.org\/10.3390\/ijms21165724","journal-title":"Int J Mol Sci"},{"issue":"2","key":"2734_CR93","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1007\/s00401-008-0396-9","volume":"116","author":"F Mori","year":"2008","unstructured":"Mori F, Tanji K, Zhang HX, Nishihira Y, Tan CF, Takahashi H et al (2008) Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia. Acta Neuropathol 116(2):193\u2013203. https:\/\/doi.org\/10.1007\/s00401-008-0396-9","journal-title":"Acta Neuropathol"},{"issue":"6","key":"2734_CR94","doi-asserted-by":"publisher","first-page":"766","DOI":"10.1016\/j.stem.2023.04.017","volume":"30","author":"Morimoto S, Takahashi S, Ito D, Dat\u00e9 Y, Okada K, Kato C, Nakamura S, Ozawa F, Chyi CM, Nishiyama A, Suzuki N, Fujimori K, Kondo T, Takao M, Hirai M, Kabe Y, Suematsu M, Jinzaki M, Aoki M, Fujiki Y, Sato Y, Suzuki N, Nakahara J; Pooled Resource Open-Access ALS Clinical Trials Consortium; Okano H","year":"2023","unstructured":"Morimoto S, Takahashi S, Ito D, Dat\u00e9 Y, Okada K, Kato C, Nakamura S, Ozawa F, Chyi CM, Nishiyama A, Suzuki N, Fujimori K, Kondo T, Takao M, Hirai M, Kabe Y, Suematsu M, Jinzaki M, Aoki M, Fujiki Y, Sato Y, Suzuki N, Nakahara J; Pooled Resource Open-Access ALS Clinical Trials Consortium; Okano H (2023) Phase 1\/2a clinical trial in ALS with ropinirole, a drug candidate identified by iPSC drug discovery. Cell Stem Cell 30(6):766-780.e9. https:\/\/doi.org\/10.1016\/j.stem.2023.04.017","journal-title":"Cell Stem Cell"},{"issue":"5","key":"2734_CR95","doi-asserted-by":"publisher","first-page":"423","DOI":"10.3390\/antiox9050423","volume":"9","author":"S Morimoto","year":"2020","unstructured":"Morimoto S, Ishikawa M, Watanabe H, Isoda M, Takao M, Nakamura S et al (2020) Brain transcriptome analysis links deficiencies of stress-responsive proteins to the pathomechanism of Kii ALS\/PDC. Antioxidants (Basel) 9(5):423. https:\/\/doi.org\/10.3390\/antiox9050423","journal-title":"Antioxidants (Basel)"},{"issue":"1","key":"2734_CR96","doi-asserted-by":"publisher","first-page":"123","DOI":"10.1002\/mds.22362","volume":"24","author":"S Morimoto","year":"2009","unstructured":"Morimoto S, Kuzuhara S, Kokubo Y (2009) Increased oxidative stress in patients with amyotrophic lateral sclerosis\/Parkinsonism-dementia complex in the Kii peninsula. Japan. Mov Disord 24(1):123\u2013126. https:\/\/doi.org\/10.1002\/mds.22362","journal-title":"Japan. Mov Disord"},{"issue":"1","key":"2734_CR97","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1042\/BJ20081386","volume":"417","author":"MP Murphy","year":"2009","unstructured":"Murphy MP (2009) How mitochondria produce reactive oxygen species. Biochem J 417(1):1\u201313. https:\/\/doi.org\/10.1042\/BJ20081386","journal-title":"Biochem J"},{"issue":"8","key":"2734_CR98","doi-asserted-by":"publisher","first-page":"1526","DOI":"10.1002\/glia.23626","volume":"67","author":"S Murru","year":"2019","unstructured":"Murru S, Hess S, Barth E, Almajan ER, Schatton D, Hermans S et al (2019) Astrocyte-specific deletion of the mitochondrial m-AAA protease reveals glial contribution to neurodegeneration. Glia 67(8):1526\u20131541. https:\/\/doi.org\/10.1002\/glia.23626","journal-title":"Glia"},{"issue":"28","key":"2734_CR99","doi-asserted-by":"publisher","first-page":"9676","DOI":"10.1074\/jbc.RA120.013478","volume":"295","author":"E Nachman","year":"2020","unstructured":"Nachman E, Wentink AS, Madiona K, Bousset L, Katsinelos T, Allinson K et al (2020) Disassembly of Tau fibrils by the human Hsp70 disaggregation machinery generates small seeding-competent species. J Biol Chem 295(28):9676\u20139690. https:\/\/doi.org\/10.1074\/jbc.RA120.013478","journal-title":"J Biol Chem"},{"issue":"5","key":"2734_CR100","doi-asserted-by":"publisher","first-page":"615","DOI":"10.1038\/nn1876","volume":"10","author":"M Nagai","year":"2007","unstructured":"Nagai M, Re DB, Nagata T, Chalazonitis A, Jessell TM, Wichterle H et al (2007) Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons. Nat Neurosci 10(5):615\u2013622. https:\/\/doi.org\/10.1038\/nn1876","journal-title":"Nat Neurosci"},{"issue":"8","key":"2734_CR101","doi-asserted-by":"publisher","first-page":"1413","DOI":"10.1242\/jcs.196469","volume":"130","author":"T Nagano","year":"2017","unstructured":"Nagano T, Nakashima A, Onishi K, Kawai K, Awai Y, Kinugasa M et al (2017) Proline dehydrogenase promotes senescence through the generation of reactive oxygen species. J Cell Sci 130(8):1413\u20131420. https:\/\/doi.org\/10.1242\/jcs.196469","journal-title":"J Cell Sci"},{"issue":"2","key":"2734_CR102","doi-asserted-by":"publisher","first-page":"137","DOI":"10.1007\/s00401-008-0477-9","volume":"117","author":"M Neumann","year":"2009","unstructured":"Neumann M, Kwong LK, Lee EB, Kremmer E, Flatley A, Xu Y et al (2009) Phosphorylation of S409\/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies. Acta Neuropathol 117(2):137\u2013149. https:\/\/doi.org\/10.1007\/s00401-008-0477-9","journal-title":"Acta Neuropathol"},{"issue":"22","key":"2734_CR103","doi-asserted-by":"publisher","first-page":"4910","DOI":"10.1523\/JNEUROSCI.1882-20.2021","volume":"41","author":"A Niewiadomska-Cimicka","year":"2021","unstructured":"Niewiadomska-Cimicka A, Doussau F, Perot JB, Roux MJ, Keime C, Hache A et al (2021) SCA7 mouse cerebellar pathology reveals preferential downregulation of key Purkinje cell-identity genes and shared disease signature with SCA1 and SCA2. J Neurosci 41(22):4910\u20134936. https:\/\/doi.org\/10.1523\/JNEUROSCI.1882-20.2021","journal-title":"J Neurosci"},{"key":"2734_CR104","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1007\/978-1-61779-452-0_3","volume":"814","author":"NA Oberheim","year":"2012","unstructured":"Oberheim NA, Goldman SA, Nedergaard M (2012) Heterogeneity of astrocytic form and function. Methods Mol Biol 814:23\u201345. https:\/\/doi.org\/10.1007\/978-1-61779-452-0_3","journal-title":"Methods Mol Biol"},{"issue":"7","key":"2734_CR105","doi-asserted-by":"publisher","first-page":"22","DOI":"10.1186\/1756-6606-7-22","volume":"31","author":"H Okano","year":"2014","unstructured":"Okano H, Yamanaka S (2014) iPS cell technologies: significance and applications to CNS regeneration and disease. Mol Brain 31(7):22. https:\/\/doi.org\/10.1186\/1756-6606-7-22","journal-title":"Mol Brain"},{"issue":"2","key":"2734_CR106","doi-asserted-by":"publisher","first-page":"99","DOI":"10.1016\/j.tips.2019.12.002","volume":"41","author":"H Okano","year":"2020","unstructured":"Okano H, Yasuda D, Fujimori K, Morimoto S, Takahashi S (2020) Ropinirole, a new ALS drug candidate developed using iPSCs. Trends Pharmacol Sci 41(2):99\u2013109. https:\/\/doi.org\/10.1016\/j.tips.2019.12.002","journal-title":"Trends Pharmacol Sci"},{"issue":"2","key":"2734_CR107","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1016\/j.stem.2022.01.007","volume":"29","author":"H Okano","year":"2022","unstructured":"Okano H, Morimoto S (2022) iPSC-based disease modeling and drug discovery in cardinal neurodegenerative disorders. Cell Stem Cell 29(2):189\u2013208. https:\/\/doi.org\/10.1016\/j.stem.2022.01.007","journal-title":"Cell Stem Cell"},{"issue":"1","key":"2734_CR108","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1007\/s12031-007-9027-5","volume":"35","author":"JL Orthmann-Murphy","year":"2008","unstructured":"Orthmann-Murphy JL, Abrams CK, Scherer SS (2008) Gap junctions couple astrocytes and oligodendrocytes. J Mol Neurosci 35(1):101\u2013116. https:\/\/doi.org\/10.1007\/s12031-007-9027-5","journal-title":"J Mol Neurosci"},{"issue":"6","key":"2734_CR109","doi-asserted-by":"publisher","first-page":"711","DOI":"10.1016\/j.beem.2012.05.003","volume":"26","author":"LD Osellame","year":"2012","unstructured":"Osellame LD, Blacker TS, Duchen MR (2012) Cellular and molecular mechanisms of mitochondrial function. Best Pract Res Clin Endocrinol Metab 26(6):711\u2013723. https:\/\/doi.org\/10.1016\/j.beem.2012.05.003","journal-title":"Best Pract Res Clin Endocrinol Metab"},{"issue":"4","key":"2734_CR110","doi-asserted-by":"publisher","first-page":"e19239","DOI":"10.1371\/journal.pone.0019239","volume":"6","author":"N Osterberg","year":"2011","unstructured":"Osterberg N, Wiehle M, Oehlke O, Heidrich S, Xu C, Fan CM et al (2011) Sim1 is a novel regulator in the differentiation of mouse dorsal raphe serotonergic neurons. PLoS ONE 6(4):e19239. https:\/\/doi.org\/10.1371\/journal.pone.0019239","journal-title":"PLoS ONE"},{"issue":"3","key":"2734_CR111","doi-asserted-by":"publisher","first-page":"308","DOI":"10.1097\/00005072-199703000-00010","volume":"56","author":"K Oyanagi","year":"1997","unstructured":"Oyanagi K, Makifuchi T, Ohtoh T, Chen KM, Gajdusek DC, Chase TN (1997) Distinct pathological features of the gallyas- and tau-positive glia in the Parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam. J Neuropathol Exp Neurol 56(3):308\u2013316. https:\/\/doi.org\/10.1097\/00005072-199703000-00010","journal-title":"J Neuropathol Exp Neurol"},{"issue":"6","key":"2734_CR112","first-page":"522","volume":"55","author":"S Ozaki","year":"2007","unstructured":"Ozaki S, Beppu H, Sonoda S, Okazaki H, Mizutani K, Itani Y et al (2007) Relationship between cytokine concentration and activities of daily living in rehabilitation patients with stroke. Rinsho Byori 55(6):522\u2013527","journal-title":"Rinsho Byori"},{"issue":"3","key":"2734_CR113","doi-asserted-by":"publisher","first-page":"380","DOI":"10.1038\/s12276-020-0399-8","volume":"52","author":"MH Park","year":"2020","unstructured":"Park MH, Jin HK, Bae JS (2020) Potential therapeutic target for aging and age-related neurodegenerative diseases: the role of acid sphingomyelinase. Exp Mol Med 52(3):380\u2013389. https:\/\/doi.org\/10.1038\/s12276-020-0399-8","journal-title":"Exp Mol Med"},{"issue":"4","key":"2734_CR114","doi-asserted-by":"publisher","first-page":"417","DOI":"10.1038\/nmeth.4197","volume":"14","author":"R Patro","year":"2017","unstructured":"Patro R, Duggal G, Love MI, Irizarry RA, Kingsford C (2017) Salmon provides fast and bias-aware quantification of transcript expression. Nat Methods 14(4):417\u2013419. https:\/\/doi.org\/10.1038\/nmeth.4197","journal-title":"Nat Methods"},{"issue":"3","key":"2734_CR115","doi-asserted-by":"publisher","first-page":"323","DOI":"10.1007\/s00401-015-1513-1","volume":"131","author":"M Pekny","year":"2016","unstructured":"Pekny M, Pekna M, Messing A, Steinh\u00e4user C, Lee JM, Parpura V et al (2016) Astrocytes: a central element in neurological diseases. Acta Neuropathol 131(3):323\u2013345. https:\/\/doi.org\/10.1007\/s00401-015-1513-1","journal-title":"Acta Neuropathol"},{"issue":"22","key":"2734_CR116","doi-asserted-by":"publisher","first-page":"10625","DOI":"10.1073\/pnas.91.22.10625","volume":"91","author":"L Pellerin","year":"1994","unstructured":"Pellerin L, Magistretti PJ (1994) Glutamate uptake into astrocytes stimulates aerobic glycolysis: a mechanism coupling neuronal activity to glucose utilization. Proc Natl Acad Sci USA 91(22):10625\u201310629. https:\/\/doi.org\/10.1073\/pnas.91.22.10625","journal-title":"Proc Natl Acad Sci USA"},{"issue":"40","key":"2734_CR117","doi-asserted-by":"publisher","first-page":"34690","DOI":"10.1074\/jbc.M111.261321","volume":"286","author":"S Pemberton","year":"2011","unstructured":"Pemberton S, Madiona K, Pieri L, Kabani M, Bousset L, Melki R (2011) Hsc70 protein interaction with soluble and fibrillar alpha-synuclein. J Biol Chem 286(40):34690\u201334699. https:\/\/doi.org\/10.1074\/jbc.M111.261321","journal-title":"J Biol Chem"},{"issue":"46","key":"2734_CR118","doi-asserted-by":"publisher","first-page":"29101","DOI":"10.1073\/pnas.2007806117","volume":"117","author":"AYT Peng","year":"2020","unstructured":"Peng AYT, Agrawal I, Ho WY, Yen YC, Pinter AJ, Liu J et al (2020) Loss of TDP-43 in astrocytes leads to motor deficits by triggering A1-like reactive phenotype and triglial dysfunction. Proc Natl Acad Sci 117(46):29101\u201329112. https:\/\/doi.org\/10.1073\/pnas.2007806117","journal-title":"Proc Natl Acad Sci"},{"issue":"6","key":"2734_CR119","doi-asserted-by":"publisher","first-page":"a020628","DOI":"10.1101\/cshperspect.a020628","volume":"7","author":"H Phatnani","year":"2015","unstructured":"Phatnani H, Maniatis T (2015) Astrocytes in neurodegenerative disease. Cold Spring Harb Perspect Biol 7(6):a020628. https:\/\/doi.org\/10.1101\/cshperspect.a020628","journal-title":"Cold Spring Harb Perspect Biol"},{"issue":"3","key":"2734_CR120","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1016\/S1474-4422(11)70015-1","volume":"10","author":"T Philips","year":"2011","unstructured":"Philips T, Robberecht W (2011) Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease. Lancet Neurol 10(3):253\u2013263. https:\/\/doi.org\/10.1016\/S1474-4422(11)70015-1","journal-title":"Lancet Neurol"},{"issue":"1","key":"2734_CR121","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1002\/mds.26078","volume":"30","author":"W Poewe","year":"2015","unstructured":"Poewe W, Antonini A (2015) Novel formulations and modes of delivery of levodopa. Mov Disord 30(1):114\u2013120. https:\/\/doi.org\/10.1002\/mds.26078","journal-title":"Mov Disord"},{"issue":"51","key":"2734_CR122","doi-asserted-by":"publisher","first-page":"e2306767120","DOI":"10.1073\/pnas.2306767120","volume":"120","author":"C Qi","year":"2023","unstructured":"Qi C, Verheijen BM, Kokubo Y, Shi Y, Tetter S, Murzin AG et al (2023) Tau filaments from amyotrophic lateral sclerosis\/parkinsonism-dementia complex adopt the CTE fold. Proc Natl Acad Sci USA 120(51):e2306767120. https:\/\/doi.org\/10.1073\/pnas.2306767120","journal-title":"Proc Natl Acad Sci USA"},{"issue":"4\u20136","key":"2734_CR123","doi-asserted-by":"publisher","first-page":"315","DOI":"10.3109\/15419060109080745","volume":"8","author":"JE Rash","year":"2001","unstructured":"Rash JE, Yasumura T, Davidson KG, Furman CS, Dudek FE, Nagy JI (2001) Identification of cells expressing Cx43, Cx30, Cx26, Cx32 and Cx36 in gap junctions of rat brain and spinal cord. Cell Commun Adhes 8(4\u20136):315\u2013320. https:\/\/doi.org\/10.3109\/15419060109080745","journal-title":"Cell Commun Adhes"},{"issue":"W1","key":"2734_CR124","doi-asserted-by":"publisher","first-page":"W191","DOI":"10.1093\/nar\/gkz369","volume":"47","author":"U Raudvere","year":"2019","unstructured":"Raudvere U, Kolberg L, Kuzmin I, Arak T, Adler P, Peterson H et al (2019) g:Profiler: a web server for functional enrichment analysis and conversions of gene lists (2019 update). Nucleic Acids Res 47(W1):W191\u2013W198. https:\/\/doi.org\/10.1093\/nar\/gkz369","journal-title":"Nucleic Acids Res"},{"issue":"2","key":"2734_CR125","doi-asserted-by":"publisher","first-page":"482","DOI":"10.1038\/s41596-018-0103-9","volume":"14","author":"J Reimand","year":"2019","unstructured":"Reimand J, Isserlin R, Voisin V, Kucera M, Tannus-Lopes C, Rostamianfar A et al (2019) Pathway enrichment analysis and visualization of omics data using g: Profiler, GSEA. Cytoscape and EnrichmentMap Nat Protoc 14(2):482\u2013517. https:\/\/doi.org\/10.1038\/s41596-018-0103-9","journal-title":"Cytoscape and EnrichmentMap Nat Protoc"},{"issue":"208","key":"2734_CR126","doi-asserted-by":"publisher","first-page":"175","DOI":"10.1016\/j.lfs.2018.07.033","volume":"1","author":"R Ren","year":"2018","unstructured":"Ren R, Zhang L, Wang M (2018) Specific deletion connexin43 in astrocyte ameliorates cognitive dysfunction in APP\/PS1 mice. Life Sci 1(208):175\u2013191. https:\/\/doi.org\/10.1016\/j.lfs.2018.07.033","journal-title":"Life Sci"},{"issue":"7\u20138","key":"2734_CR127","doi-asserted-by":"publisher","first-page":"457","DOI":"10.1016\/s0248-4900(02)00016-3","volume":"94","author":"N Rouach","year":"2022","unstructured":"Rouach N, Avignone E, M\u00eame W, Koulakoff A, Venance L, Blomstrand F et al (2022) Gap junctions and connexin expression in the normal and pathological central nervous system. Biol Cell 94(7\u20138):457\u2013475. https:\/\/doi.org\/10.1016\/s0248-4900(02)00016-3","journal-title":"Biol Cell"},{"issue":"22","key":"2734_CR128","doi-asserted-by":"publisher","first-page":"e2437","DOI":"10.1212\/WNL.0000000000201156","volume":"99","author":"R Sasaki","year":"2022","unstructured":"Sasaki R, Morimoto S, Ozawa F, Okano H, Yoshida M, Ishiura H et al (2022) Neurology 99(22):e2437\u2013e2442. https:\/\/doi.org\/10.1212\/WNL.0000000000201156","journal-title":"Neurology"},{"issue":"9","key":"2734_CR129","doi-asserted-by":"publisher","first-page":"655","DOI":"10.1038\/nrm2959","volume":"11","author":"O Schmidt","year":"2010","unstructured":"Schmidt O, Pfanner N, Meisinger C (2010) Mitochondrial protein import: from proteomics to functional mechanisms. Nat Rev Mol Cell Biol 11(9):655\u2013667. https:\/\/doi.org\/10.1038\/nrm2959","journal-title":"Nat Rev Mol Cell Biol"},{"issue":"2","key":"2734_CR130","doi-asserted-by":"publisher","first-page":"464","DOI":"10.1096\/fj.09-137562","volume":"24","author":"M Seo","year":"2010","unstructured":"Seo M, Lee WH, Suk K (2010) Identification of novel cell migration-promoting genes by a functional genetic screen. FASEB J 24(2):464\u2013478. https:\/\/doi.org\/10.1096\/fj.09-137562","journal-title":"FASEB J"},{"key":"2734_CR131","doi-asserted-by":"publisher","first-page":"217.e9","DOI":"10.1016\/j.neurobiolaging.2015.10.040","volume":"38","author":"CH Shi","year":"2016","unstructured":"Shi CH, Mao CY, Zhang SY, Yang J, Song B, Wu P et al (2016) CHCHD2 gene mutations in familial and sporadic Parkinson\u2019s disease. Neurobiol Aging 38:217.e9-217.e13. https:\/\/doi.org\/10.1016\/j.neurobiolaging.2015.10.040","journal-title":"Neurobiol Aging"},{"issue":"13","key":"2734_CR132","doi-asserted-by":"publisher","first-page":"29","DOI":"10.3389\/fncir.2019.00029","volume":"8","author":"S Shibata","year":"2019","unstructured":"Shibata S, Iseda T, Mitsuhashi T, Oka A, Shindo T, Moritoki N et al (2019) Large-area fluorescence and electron microscopic correlative imaging with multibeam scanning electron microscopy. Front Neural Circuits 8(13):29. https:\/\/doi.org\/10.3389\/fncir.2019.00029","journal-title":"Front Neural Circuits"},{"issue":"2","key":"2734_CR133","doi-asserted-by":"publisher","first-page":"e136","DOI":"10.1212\/WNL.0000000000006736","volume":"92","author":"H Shinotoh","year":"2019","unstructured":"Shinotoh H, Shimada H, Kokubo Y, Tagai K, Niwa F, Kitamura S et al (2019) Tau imaging detects distinctive distribution of tau pathology in ALS\/PDC on the Kii Peninsula. Neurology 92(2):e136\u2013e147. https:\/\/doi.org\/10.1212\/WNL.0000000000006736","journal-title":"Neurology"},{"issue":"4","key":"2734_CR134","doi-asserted-by":"publisher","first-page":"974","DOI":"10.1111\/j.1471-4159.2005.03428.x","volume":"95","author":"RM Silva","year":"2005","unstructured":"Silva RM, Ries V, Oo TF, Yarygina O, Jackson-Lewis V, Ryu EJ et al (2005) CHOP\/GADD153 is a mediator of apoptotic death in substantia nigra dopamine neurons in an in vivo neurotoxin model of parkinsonism. J Neurochem 95(4):974\u2013986. https:\/\/doi.org\/10.1111\/j.1471-4159.2005.03428.x","journal-title":"J Neurochem"},{"key":"2734_CR135","doi-asserted-by":"publisher","first-page":"1521","DOI":"10.12688\/f1000research.7563.1","volume":"4","author":"C Soneson","year":"2015","unstructured":"Soneson C, Love MI, Robinson MD (2015) Differential analyses for RNA-seq: transcript level estimates improve gene level inferences. F1000Res 4:1521. https:\/\/doi.org\/10.12688\/f1000research.7563.1","journal-title":"F1000Res"},{"issue":"6645","key":"2734_CR136","doi-asserted-by":"publisher","first-page":"839","DOI":"10.1038\/42166","volume":"388","author":"MG Spillantini","year":"1997","unstructured":"Spillantini MG, Schmidt ML, Lee VM, Trojanowski JQ, Jakes R, Goedert M (1997) Alpha-synuclein in Lewy bodies. Nature 388(6645):839\u2013840. https:\/\/doi.org\/10.1038\/42166","journal-title":"Nature"},{"issue":"1","key":"2734_CR137","doi-asserted-by":"publisher","first-page":"111942","DOI":"10.1016\/j.celrep.2022.111942","volume":"42","author":"J Sun","year":"2023","unstructured":"Sun J, Osenberg S, Irwin A, Ma LH, Lee N, Xiang Y et al (2023) Mutations in the transcriptional regulator MeCP2 severely impact key cellular and molecular signatures of human astrocytes during maturation. Cell Rep 42(1):111942. https:\/\/doi.org\/10.1016\/j.celrep.2022.111942","journal-title":"Cell Rep"},{"issue":"26","key":"2734_CR138","doi-asserted-by":"publisher","first-page":"25216","DOI":"10.1074\/jbc.M503341200","volume":"280","author":"JY Sung","year":"2005","unstructured":"Sung JY, Park SM, Lee CH, Um JW, Lee HJ, Kim J et al (2005) Proteolytic cleavage of extracellular secreted {alpha}-synuclein via matrix metalloproteinases. J Biol Chem 280(26):25216\u201325224. https:\/\/doi.org\/10.1074\/jbc.M503341200","journal-title":"J Biol Chem"},{"issue":"13","key":"2734_CR139","doi-asserted-by":"publisher","DOI":"10.3389\/fnagi.2021","volume":"4","author":"S Supakul","year":"2021","unstructured":"Supakul S, Okano H, Maeda S (2021) Utilization of human induced pluripotent stem cells-derived in vitro models for the future study of sex differences in Alzheimer\u2019s disease. Front Aging Neurosci 4(13):768948. https:\/\/doi.org\/10.3389\/fnagi.2021","journal-title":"Front Aging Neurosci"},{"key":"2734_CR140","doi-asserted-by":"publisher","DOI":"10.1016\/j.scr.2022.102802","volume":"62","author":"S Supakul","year":"2022","unstructured":"Supakul S, Leventoux N, Tabuchi H, Mimura M, Ito D, Maeda S et al (2022) Establishment of KEIOi005-A iPSC line from urine-derived cells (UDCs) of a mild Alzheimer\u2019s disease (AD) donor with multiple risk SNPs for sporadic Alzheimer\u2019s disease (sAD). Stem Cell Res 62:102802. https:\/\/doi.org\/10.1016\/j.scr.2022.102802","journal-title":"Stem Cell Res"},{"issue":"4","key":"2734_CR141","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbas.2023.100101","volume":"19","author":"S Supakul","year":"2023","unstructured":"Supakul S, Hatakeyama Y, Leventoux N, Itsuno M, Numata N, Hiramine H et al (2023) Urine-derived cells from the aged donor for the 2D\/3D modeling of neural cells via iPSCs. Aging Brain 19(4):100101. https:\/\/doi.org\/10.1016\/j.nbas.2023.100101","journal-title":"Aging Brain"},{"key":"2734_CR142","doi-asserted-by":"publisher","DOI":"10.1186\/s41232-023-00310-5","author":"S Supakul","year":"2023","unstructured":"Supakul S, Murakami R, Oyama C, Shindo T, Hatakeyama Y, Itsuno M et al (2023) Mutual interaction of neurons and astrocytes derived from iPSCs with APP V717L mutation developed the astrocytic phenotypes of Alzheimer\u2019s disease. Inflammation and Regeneration. https:\/\/doi.org\/10.1186\/s41232-023-00310-5","journal-title":"Inflammation and Regeneration"},{"issue":"5","key":"2734_CR143","doi-asserted-by":"publisher","first-page":"861","DOI":"10.1016\/j.cell.2007.11.019","volume":"131","author":"K Takahashi","year":"2007","unstructured":"Takahashi K, Tanabe K, Ohnuki M, Narita M, Ichisaka T, Tomoda K et al (2007) Induction of pluripotent stem cells from adult human fibroblasts by defined factors. Cell 131(5):861\u2013872. https:\/\/doi.org\/10.1016\/j.cell.2007.11.019","journal-title":"Cell"},{"key":"2734_CR144","doi-asserted-by":"publisher","first-page":"00","DOI":"10.5772\/intechopen.97035","volume":"01","author":"S Takahashi","year":"2021","unstructured":"Takahashi S (2021) Lactate and ketone bodies act as energy substrates as well as signal molecules in the brain. psychology and pathophysiological outcomes of eating. IntechOpen 01:00. https:\/\/doi.org\/10.5772\/intechopen.97035","journal-title":"IntechOpen"},{"issue":"2","key":"2734_CR145","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1111\/neup.12639","volume":"40","author":"S Takahashi","year":"2020","unstructured":"Takahashi S (2020) Metabolic compartmentalization between astroglia and neurons in physiological and pathophysiological conditions of the neurovascular unit. Neuropathology 40(2):121\u2013137. https:\/\/doi.org\/10.1111\/neup.12639","journal-title":"Neuropathology"},{"issue":"1","key":"2734_CR146","doi-asserted-by":"publisher","first-page":"170","DOI":"10.3390\/antiox11010170","volume":"11","author":"S Takahashi","year":"2022","unstructured":"Takahashi S, Mashima K (2022) Neuroprotection and Disease Modification by Astrocytes and Microglia in Parkinson Disease. Antioxidants (Basel) 11(1):170. https:\/\/doi.org\/10.3390\/antiox11010170","journal-title":"Antioxidants (Basel)"},{"key":"2734_CR147","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0021108","volume":"6","author":"H Takeuchi","year":"2011","unstructured":"Takeuchi H, Mizoguchi H, Doi Y, Jin S, Noda M, Liang J et al (2011) Blockade of gap junction hemichannel suppresses disease progression in mouse models of amyotrophic lateral sclerosis and Alzheimer\u2019s disease. PLoS ONE 6:e21108. https:\/\/doi.org\/10.1371\/journal.pone.0021108","journal-title":"PLoS ONE"},{"issue":"13","key":"2734_CR148","doi-asserted-by":"publisher","first-page":"1815","DOI":"10.1002\/dneu.20559","volume":"67","author":"AR Taylor","year":"2007","unstructured":"Taylor AR, Robinson MB, Gifondorwa DJ, Tytell M, Milligan CE (2007) Regulation of heat shock protein 70 release in astrocytes: role of signaling kinases. Dev Neurobiol 67(13):1815\u20131829. https:\/\/doi.org\/10.1002\/dneu.20559","journal-title":"Dev Neurobiol"},{"issue":"3","key":"2734_CR149","doi-asserted-by":"publisher","first-page":"267","DOI":"10.1038\/s41587-019-0035-0","volume":"37","author":"J Tchieu","year":"2019","unstructured":"Tchieu J, Calder EL, Guttikonda SR, Gutzwiller EM, Aromolaran KA, Steinbeck JA et al (2019) NFIA is a gliogenic switch enabling rapid derivation of functional human astrocytes from pluripotent stem cells. Nat Biotechnol 37(3):267\u2013275. https:\/\/doi.org\/10.1038\/s41587-019-0035-0","journal-title":"Nat Biotechnol"},{"issue":"4","key":"2734_CR150","doi-asserted-by":"publisher","first-page":"452","DOI":"10.1038\/nn.2778","volume":"14","author":"JR Tollervey","year":"2011","unstructured":"Tollervey JR, Curk T, Rogelj B, Briese M, Cereda M, Kayikci M et al (2011) Characterizing the RNA targets and position-dependent splicing regulation by TDP-43. Nat Neurosci 14(4):452\u2013458. https:\/\/doi.org\/10.1038\/nn.2778","journal-title":"Nat Neurosci"},{"issue":"16","key":"2734_CR151","doi-asserted-by":"publisher","first-page":"2344","DOI":"10.1002\/mds.22262","volume":"23","author":"H Tomiyama","year":"2008","unstructured":"Tomiyama H, Kokubo Y, Sasaki R, Li Y, Imamichi Y, Funayama M et al (2008) Mutation analyses in amyotrophic lateral sclerosis\/parkinsonism-dementia complex of the Kii peninsula. Japan. Mov Disord 23(16):2344\u20132348. https:\/\/doi.org\/10.1002\/mds.22262","journal-title":"Japan. Mov Disord"},{"issue":"1","key":"2734_CR152","doi-asserted-by":"publisher","first-page":"4147","DOI":"10.1038\/s41467-019-12099-4","volume":"10","author":"T Vandoorne","year":"2019","unstructured":"Vandoorne T, Veys K, Guo W, Sicart A, Vints K, Swijsen A et al (2019) Differentiation but not ALS mutations in FUS rewires motor neuron metabolism. Nat Commun 10(1):4147. https:\/\/doi.org\/10.1038\/s41467-019-12099-4","journal-title":"Nat Commun"},{"issue":"1","key":"2734_CR153","doi-asserted-by":"publisher","first-page":"6003","DOI":"10.1038\/s41598-020-62864-5","volume":"10","author":"J Velebit","year":"2020","unstructured":"Velebit J, Horvat A, Smoli\u010d T, Prpar Mihevc S, Rogelj B, Zorec R et al (2020) Astrocytes with TDP-43 inclusions exhibit reduced noradrenergic cAMP and Ca2+\u00a0signaling and dysregulated cell metabolism. Sci Rep 10(1):6003. https:\/\/doi.org\/10.1038\/s41598-020-62864-5","journal-title":"Sci Rep"},{"issue":"8","key":"2734_CR154","doi-asserted-by":"publisher","first-page":"902","DOI":"10.1093\/jnen\/nlaa056","volume":"79","author":"BM Verheijen","year":"2020","unstructured":"Verheijen BM, Morimoto S, Sasaki R, Oyanagi K, Kokubo Y, Kuzuhara S et al (2020) Expression of mutant ubiquitin and proteostasis impairment in Kii amyotrophic lateral sclerosis\/parkinsonism-dementia complex brains. J Neuropathol Exp Neurol 79(8):902\u2013907. https:\/\/doi.org\/10.1093\/jnen\/nlaa056","journal-title":"J Neuropathol Exp Neurol"},{"issue":"19","key":"2734_CR155","doi-asserted-by":"publisher","first-page":"6448","DOI":"10.1523\/JNEUROSCI.0248-14.2014","volume":"34","author":"AK Walker","year":"2014","unstructured":"Walker AK, Daniels CM, Goldman JE, Trojanowski JQ, Lee VM, Messing A (2014) Astrocytic TDP-43 pathology in Alexander disease. J Neurosci 34(19):6448\u20136458. https:\/\/doi.org\/10.1523\/JNEUROSCI.0248-14.2014","journal-title":"J Neurosci"},{"issue":"21","key":"2734_CR156","doi-asserted-by":"publisher","first-page":"2753","DOI":"10.1093\/hmg\/ddg312","volume":"12","author":"J Wang","year":"2003","unstructured":"Wang J, Slunt H, Gonzales V, Fromholt D, Coonfield M, Copeland NG et al (2003) Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature. Hum Mol Genet 12(21):2753\u20132764. https:\/\/doi.org\/10.1093\/hmg\/ddg312","journal-title":"Hum Mol Genet"},{"issue":"4","key":"2734_CR157","doi-asserted-by":"publisher","first-page":"586","DOI":"10.1016\/j.neurobiolaging.2006.11.009","volume":"29","author":"J Wang","year":"2008","unstructured":"Wang J, Martin E, Gonzales V, Borchelt DR, Lee MK (2008) Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases. Neurobiol Aging 29(4):586\u2013597. https:\/\/doi.org\/10.1016\/j.neurobiolaging.2006.11.009","journal-title":"Neurobiol Aging"},{"issue":"2","key":"2734_CR158","doi-asserted-by":"publisher","first-page":"585","DOI":"10.3892\/mmr.2013.1535","volume":"8","author":"Y Wang","year":"2013","unstructured":"Wang Y, Wu Z, Liu X, Fu Q (2013) Gastrodin ameliorates Parkinson\u2019s disease by downregulating connexin 43. Mol Med Rep 8(2):585\u2013590. https:\/\/doi.org\/10.3892\/mmr.2013.1535","journal-title":"Mol Med Rep"},{"issue":"2","key":"2734_CR159","doi-asserted-by":"publisher","first-page":"0500","DOI":"10.1523\/ENEURO.0500-20.2021","volume":"8","author":"H Watanabe","year":"2021","unstructured":"Watanabe H, Imaizumi K, Cai T, Zhou Z, Tomita T, Okano T (2021) Flexible and accurate substrate processing with distinct presenilin\/\u03b3-secretases in human cortical neurons. eNeuro 8(2):0500\u20130520. https:\/\/doi.org\/10.1523\/ENEURO.0500-20.2021","journal-title":"eNeuro"},{"issue":"3","key":"2734_CR160","doi-asserted-by":"publisher","first-page":"1139","DOI":"10.1172\/JCI130988","volume":"130","author":"K Weskamp","year":"2020","unstructured":"Weskamp K, Tank EM, Miguez R, McBride JP, G\u00f3mez NB, White M et al (2020) Shortened TDP43 isoforms upregulated by neuronal hyperactivity drive TDP43 pathology in ALS. J Clin Invest 130(3):1139\u20131155. https:\/\/doi.org\/10.1172\/JCI130988","journal-title":"J Clin Invest"},{"issue":"5","key":"2734_CR161","doi-asserted-by":"publisher","first-page":"2408","DOI":"10.1021\/acschemneuro.8b00704","volume":"10","author":"B Xu","year":"2019","unstructured":"Xu B, Zheng C, Chen X, Zhang Z, Liu J, Spencer P et al (2019) Dysregulation of Myosin Complex and Striated Muscle Contraction Pathway in the Brains of ALS-SOD1 Model Mice. ACS Chem Neurosci 10(5):2408\u20132417. https:\/\/doi.org\/10.1021\/acschemneuro.8b00704","journal-title":"ACS Chem Neurosci"},{"issue":"3","key":"2734_CR162","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1038\/nn2047","volume":"11","author":"K Yamanaka","year":"2008","unstructured":"Yamanaka K, Chun SJ, Boillee S, Fujimori-Tonou N, Yamashita H, Gutmann DH et al (2008) Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat Neurosci 11(3):251\u2013253. https:\/\/doi.org\/10.1038\/nn2047","journal-title":"Nat Neurosci"},{"issue":"10","key":"2734_CR163","doi-asserted-by":"publisher","first-page":"839","DOI":"10.1097\/01.jnen.0000182977.79483.89","volume":"64","author":"M Yamazaki","year":"2005","unstructured":"Yamazaki M, Hasegawa M, Mori O, Murayama S, Tsuchiya K, Ikeda K et al (2005) Tau-positive fine granules in the cerebral white matter: a novel finding among the tauopathies exclusive to parkinsonism-dementia complex of Guam. J Neuropathol Exp Neurol 64(10):839\u2013846. https:\/\/doi.org\/10.1097\/01.jnen.0000182977.79483.89","journal-title":"J Neuropathol Exp Neurol"},{"issue":"2","key":"2734_CR164","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1159\/000116800","volume":"32","author":"M Yasui","year":"1992","unstructured":"Yasui M, Yase Y, Kihira T, Adachi K, Suzuki Y (1992) Magnesium and calcium contents in CNS tissues of amyotrophic lateral sclerosis patients from the Kii peninsula. Japan Eur Neurol 32(2):95\u201398. https:\/\/doi.org\/10.1159\/000116800","journal-title":"Japan Eur Neurol"},{"issue":"1","key":"2734_CR165","first-page":"39","volume":"10","author":"M Yasui","year":"1997","unstructured":"Yasui M, Ota K, Yoshida M (1997) Effects of low calcium and magnesium dietary intake on the central nervous system tissues of rats and calcium-magnesium related disorders in the amyotrophic lateral sclerosis focus in the Kii Peninsula of Japan. Magnes Res 10(1):39\u201350 (PMID: 9339837)","journal-title":"Magnes Res"},{"issue":"1265","key":"2734_CR166","doi-asserted-by":"publisher","first-page":"158","DOI":"10.1016\/j.brainres.2009.01.058","volume":"10","author":"GN Yin","year":"2009","unstructured":"Yin GN, Lee HW, Cho JY, Suk K (2009) Neuronal pentraxin receptor in cerebrospinal fluid as a potential biomarker for neurodegenerative diseases. Brain Res 10(1265):158\u2013170. https:\/\/doi.org\/10.1016\/j.brainres.2009.01.058","journal-title":"Brain Res"},{"key":"2734_CR167","doi-asserted-by":"publisher","first-page":"58","DOI":"10.1016\/j.mcn.2017.11.013","volume":"86","author":"WW Yu","year":"2018","unstructured":"Yu WW, Cao SN, Zang CX, Wang L, Yang HY, Bao XQ et al (2018) Heat shock protein 70 suppresses neuroinflammation induced by \u03b1-synuclein in astrocytes. Mol Cell Neurosci 86:58\u201364. https:\/\/doi.org\/10.1016\/j.mcn.2017.11.013","journal-title":"Mol Cell Neurosci"},{"issue":"10","key":"2734_CR168","doi-asserted-by":"publisher","first-page":"633","DOI":"10.1089\/scd.2019.0033","volume":"28","author":"W Yun","year":"2019","unstructured":"Yun W, Hong W, Son D, Liu HW, Kim SS, Park M et al (2019) Generation of anterior hindbrain-specific, glial-restricted progenitor-like cells from human pluripotent stem cells. Stem Cells Dev 28(10):633\u2013648. https:\/\/doi.org\/10.1089\/scd.2019.0033","journal-title":"Stem Cells Dev"},{"issue":"1","key":"2734_CR169","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1016\/j.neuron.2015.11.013","volume":"89","author":"Y Zhang","year":"2016","unstructured":"Zhang Y, Sloan SA, Clarke LE, Caneda C, Plaza CA, Blumenthal PD et al (2016) Purification and characterization of progenitor and mature human astrocytes reveals transcriptional and functional differences with mouse. Neuron 89(1):37\u201353. https:\/\/doi.org\/10.1016\/j.neuron.2015.11.013","journal-title":"Neuron"},{"issue":"6","key":"2734_CR170","doi-asserted-by":"publisher","first-page":"4699","DOI":"10.1007\/s11033-022-07319-y","volume":"49","author":"Y Zhang","year":"2022","unstructured":"Zhang Y, Yang X, Zhuang J, Zhang H, Gao C (2022) \u03b2-Amyloid activates reactive astrocytes by enhancing glycolysis of astrocytes. Mol Biol Rep 49(6):4699\u20134707. https:\/\/doi.org\/10.1007\/s11033-022-07319-y","journal-title":"Mol Biol Rep"},{"issue":"1","key":"2734_CR171","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1186\/s12974-019-1627-9","volume":"16","author":"W Zhao","year":"2019","unstructured":"Zhao W, Xu Z, Cao J, Fu Q, Wu Y, Zhang X et al (2019) Elamipretide (SS-31) improves mitochondrial dysfunction, synaptic and memory impairment induced by lipopolysaccharide in mice. J Neuroinflammation 16(1):230. https:\/\/doi.org\/10.1186\/s12974-019-1627-9","journal-title":"J Neuroinflammation"},{"issue":"6","key":"2734_CR172","doi-asserted-by":"publisher","first-page":"665","DOI":"10.1111\/cns.13123","volume":"25","author":"B Zhou","year":"2019","unstructured":"Zhou B, Zuo YX, Jiang RT (2019) Astrocyte morphology: Diversity, plasticity, and role in neurological diseases. CNS Neurosci Ther 25(6):665\u2013673. https:\/\/doi.org\/10.1111\/cns.13123","journal-title":"CNS Neurosci Ther"},{"issue":"7","key":"2734_CR173","doi-asserted-by":"publisher","first-page":"1100","DOI":"10.1093\/hmg\/ddy413","volume":"28","author":"W Zhou","year":"2019","unstructured":"Zhou W, Ma D, Sun AX, Tran HD, Ma DL, Singh BK et al (2019) PD-linked CHCHD2 mutations impair CHCHD10 and MICOS complex leading to mitochondria dysfunction. Hum Mol Genet 28(7):1100\u20131116. https:\/\/doi.org\/10.1093\/hmg\/ddy413","journal-title":"Hum Mol Genet"},{"issue":"2","key":"2734_CR174","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1083\/jcb.201601061","volume":"215","author":"L Zhu","year":"2016","unstructured":"Zhu L, Gomez-Duran A, Saretzki G, Jin S, Tilgner K, Melguizo-Sanchis D et al (2016) The mitochondrial protein CHCHD2 primes the differentiation potential of human induced pluripotent stem cells to neuroectodermal lineages. J Cell Biol 215(2):187\u2013202. https:\/\/doi.org\/10.1083\/jcb.201601061","journal-title":"J Cell Biol"},{"issue":"1","key":"2734_CR175","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1101\/gr.275939.121","volume":"32","author":"OJ Ziff","year":"2022","unstructured":"Ziff OJ, Clarke BE, Taha DM, Crerar H, Luscombe NM, Patani R (2022) Meta-analysis of human and mouse ALS astrocytes reveals multi-omic signatures of inflammatory reactive states. Genome Res 32(1):71\u201384. https:\/\/doi.org\/10.1101\/gr.275939.121","journal-title":"Genome Res"}],"container-title":["Acta Neuropathologica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-024-02734-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00401-024-02734-w\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-024-02734-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,24]],"date-time":"2024-05-24T15:04:13Z","timestamp":1716563053000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00401-024-02734-w"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,15]]},"references-count":175,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2024,6]]}},"alternative-id":["2734"],"URL":"https:\/\/doi.org\/10.1007\/s00401-024-02734-w","relation":{},"ISSN":["0001-6322","1432-0533"],"issn-type":[{"value":"0001-6322","type":"print"},{"value":"1432-0533","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5,15]]},"assertion":[{"value":"13 October 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 February 2024","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 April 2024","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 May 2024","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 May 2024","order":5,"name":"change_date","label":"Change Date","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Update","order":6,"name":"change_type","label":"Change Type","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The original article has been corrected. \u201cKaryotype analysis\u201d section head corrected to level 2 heading","order":7,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"H.O. reports grants and personal fees from K Pharma, Inc. during the conduct of the study; personal fees from Sanbio Co. Ltd., outside the submitted work; In addition, H.O. has a patent on a therapeutic agent for amyotrophic lateral sclerosis and composition for treatment licensed to K Pharma, Inc.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest\u00a0"}}],"article-number":"84"}}