{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,10]],"date-time":"2026-11-10T08:43:27Z","timestamp":1794300207434,"version":"4.9.0"},"reference-count":54,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2022,6,7]],"date-time":"2022-06-07T00:00:00Z","timestamp":1654560000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2022,6,7]],"date-time":"2022-06-07T00:00:00Z","timestamp":1654560000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100012166","name":"MOST|National Key Research and Development Program of China","doi-asserted-by":"publisher","award":["2021YFA1101802"],"award-info":[{"award-number":["2021YFA1101802"]}],"id":[{"id":"10.13039\/501100012166","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100012166","name":"MOST|National Key Research and Development Program of China","doi-asserted-by":"publisher","award":["2019YFA0802703"],"award-info":[{"award-number":["2019YFA0802703"]}],"id":[{"id":"10.13039\/501100012166","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82022021"],"award-info":[{"award-number":["82022021"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81971189"],"award-info":[{"award-number":["81971189"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81922022"],"award-info":[{"award-number":["81922022"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["91849117"],"award-info":[{"award-number":["91849117"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005065","name":"Jiangsu Province Innovative and Entrepreneurial Team","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005065","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["EMBO Mol Med"],"published-print":{"date-parts":[[2022,7,7]]},"DOI":"10.15252\/emmm.202215851","type":"journal-article","created":{"date-parts":[[2022,6,7]],"date-time":"2022-06-07T03:56:29Z","timestamp":1654574189000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":74,"title":["Mitochondrial HSF1 triggers mitochondrial dysfunction and neurodegeneration in Huntington's disease"],"prefix":"10.1038","volume":"14","author":[{"given":"Chunyue","family":"Liu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zixing","family":"Fu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shanshan","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaosong","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shengrong","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chu","family":"Chu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuan","family":"Hong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wenbo","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shengqi","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yueqing","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6069-3553","authenticated-orcid":false,"given":"Yang","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yongbo","family":"Song","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2918-5398","authenticated-orcid":false,"given":"Yan","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0216-0310","authenticated-orcid":false,"given":"Xing","family":"Guo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,6,7]]},"reference":[{"key":"BFEMMM202215851_CR1","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1038\/nrm2938","volume":"11","author":"M Akerfelt","year":"2010","unstructured":"Akerfelt M, Morimoto RI, Sistonen L (2010) Heat shock factors: integrators of cell stress, development and lifespan. Nat Rev Mol Cell Biol 11: 545\u2013555","journal-title":"Nat Rev Mol Cell Biol"},{"key":"BFEMMM202215851_CR2","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1002\/ana.410270403","volume":"27","author":"RL Albin","year":"1990","unstructured":"Albin RL, Reiner A, Anderson KD, Penney JB, Young AB (1990) Striatal and nigral neuron subpopulations in rigid Huntington's disease: implications for the functional anatomy of chorea and rigidity\u2010akinesia. Ann Neurol 27: 357\u2013365","journal-title":"Ann Neurol"},{"key":"BFEMMM202215851_CR3","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1016\/j.neuron.2018.10.007","volume":"100","author":"ND Amin","year":"2018","unstructured":"Amin ND, Pasca SP (2018) Building models of brain disorders with three\u2010dimensional organoids. Neuron 100: 389\u2013405","journal-title":"Neuron"},{"key":"BFEMMM202215851_CR4","doi-asserted-by":"crossref","first-page":"1496","DOI":"10.1038\/s41593-020-00730-3","volume":"23","author":"I Chiaradia","year":"2020","unstructured":"Chiaradia I, Lancaster MA (2020) Brain organoids for the study of human neurobiology at the interface of in\u2009vitro and in\u2009vivo. Nat Neurosci 23: 1496\u20131508","journal-title":"Nat Neurosci"},{"key":"BFEMMM202215851_CR5","doi-asserted-by":"crossref","first-page":"12725","DOI":"10.1523\/JNEUROSCI.4619-08.2008","volume":"28","author":"CM Cowan","year":"2008","unstructured":"Cowan CM, Fan MM, Fan J, Shehadeh J, Zhang LY, Graham RK, Hayden MR, Raymond LA (2008) Polyglutamine\u2010modulated striatal calpain activity in YAC transgenic Huntington disease mouse model: impact on NMDA receptor function and toxicity. J Neurosci 28: 12725\u201312735","journal-title":"J Neurosci"},{"key":"BFEMMM202215851_CR6","doi-asserted-by":"crossref","first-page":"957","DOI":"10.1007\/s13311-019-00782-9","volume":"16","author":"J Creus\u2010Muncunill","year":"2019","unstructured":"Creus\u2010Muncunill J, Ehrlich ME (2019) Cell\u2010autonomous and non\u2010cell\u2010autonomous pathogenic mechanisms in Huntington's Disease: insights from in\u2009vitro and in\u2009vivo models. Neurotherapeutics 16: 957\u2013978","journal-title":"Neurotherapeutics"},{"key":"BFEMMM202215851_CR7","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1172\/JCI128514","volume":"130","author":"V Del Dotto","year":"2020","unstructured":"Del Dotto V, Ullah F, Di Meo I, Magini P, Gusic M, Maresca A, Caporali L, Palombo F, Tagliavini F, Baugh EH et\u2009al (2020) SSBP1 mutations cause mtDNA depletion underlying a complex optic atrophy disorder. J Clin Invest 130: 108\u2013125","journal-title":"J Clin Invest"},{"key":"BFEMMM202215851_CR8","doi-asserted-by":"crossref","first-page":"2655","DOI":"10.1084\/jem.20160776","volume":"213","author":"MH Disatnik","year":"2016","unstructured":"Disatnik MH, Joshi AU, Saw NL, Shamloo M, Leavitt BR, Qi X, Mochly\u2010Rosen D (2016) Potential biomarkers to follow the progression and treatment response of Huntington's disease. J Exp Med 213: 2655\u20132669","journal-title":"J Exp Med"},{"key":"BFEMMM202215851_CR9","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1146\/annurev.biochem.76.060305.152028","volume":"76","author":"M Falkenberg","year":"2007","unstructured":"Falkenberg M, Larsson NG, Gustafsson CM (2007) DNA replication and transcription in mammalian mitochondria. Annu Rev Biochem 76: 679\u2013699","journal-title":"Annu Rev Biochem"},{"key":"BFEMMM202215851_CR10","doi-asserted-by":"crossref","first-page":"9117","DOI":"10.1038\/s41598-021-88715-5","volume":"11","author":"C Gomez\u2010Paredes","year":"2021","unstructured":"Gomez\u2010Paredes C, Mason MA, Taxy BA, Papadopoulou AS, Paganetti P, Bates GP (2021) The heat shock response, determined by QuantiGene multiplex, is impaired in HD mouse models and not caused by HSF1 reduction. Sci Rep 11: 9117","journal-title":"Sci Rep"},{"key":"BFEMMM202215851_CR11","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1038\/nrm.2017.73","volume":"19","author":"R Gomez\u2010Pastor","year":"2018","unstructured":"Gomez\u2010Pastor R, Burchfiel ET, Thiele DJ (2018) Regulation of heat shock transcription factors and their roles in physiology and disease. Nat Rev Mol Cell Biol 19: 4\u201319","journal-title":"Nat Rev Mol Cell Biol"},{"key":"BFEMMM202215851_CR12","doi-asserted-by":"crossref","first-page":"2363","DOI":"10.1038\/s41380-018-0229-8","volume":"23","author":"C Gonzalez","year":"2018","unstructured":"Gonzalez C, Armijo E, Bravo\u2010Alegria J, Becerra\u2010Calixto A, Mays CE, Soto C (2018) Modeling amyloid beta and tau pathology in human cerebral organoids. Mol Psychiatry 23: 2363\u20132374","journal-title":"Mol Psychiatry"},{"key":"BFEMMM202215851_CR13","doi-asserted-by":"crossref","first-page":"5371","DOI":"10.1172\/JCI70911","volume":"123","author":"X Guo","year":"2013","unstructured":"Guo X, Disatnik MH, Monbureau M, Shamloo M, Mochly\u2010Rosen D, Qi X (2013) Inhibition of mitochondrial fragmentation diminishes Huntington's disease\u2010associated neurodegeneration. J Clin Invest 123: 5371\u20135388","journal-title":"J Clin Invest"},{"key":"BFEMMM202215851_CR14","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1042\/BJ20131438","volume":"461","author":"X Guo","year":"2014","unstructured":"Guo X, Sesaki H, Qi X (2014) Drp1 stabilizes p53 on the mitochondria to trigger necrosis under oxidative stress conditions in\u2009vitro and in\u2009vivo. Biochem J 461: 137\u2013146","journal-title":"Biochem J"},{"key":"BFEMMM202215851_CR15","doi-asserted-by":"crossref","first-page":"12646","DOI":"10.1038\/ncomms12646","volume":"7","author":"X Guo","year":"2016","unstructured":"Guo X, Sun X, Hu D, Wang YJ, Fujioka H, Vyas R, Chakrapani S, Joshi AU, Luo Y, Mochly\u2010Rosen D et\u2009al (2016) VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease. Nat Commun 7: 12646","journal-title":"Nat Commun"},{"key":"BFEMMM202215851_CR16","doi-asserted-by":"crossref","first-page":"7974","DOI":"10.1523\/JNEUROSCI.0006-07.2007","volume":"27","author":"S Homma","year":"2007","unstructured":"Homma S, Jin X, Wang G, Tu N, Min J, Yanasak N, Mivechi NF (2007) Demyelination, astrogliosis, and accumulation of ubiquitinated proteins, hallmarks of CNS disease in hsf1\u2010deficient mice. J Neurosci 27: 7974\u20137986","journal-title":"J Neurosci"},{"key":"BFEMMM202215851_CR17","doi-asserted-by":"crossref","first-page":"1879","DOI":"10.1212\/WNL.45.10.1879","volume":"45","author":"TM Horton","year":"1995","unstructured":"Horton TM, Graham BH, Corral\u2010Debrinski M, Shoffner JM, Kaufman AE, Beal MF, Wallace DC (1995) Marked increase in mitochondrial DNA deletion levels in the cerebral cortex of Huntington's disease patients. Neurology 45: 1879\u20131883","journal-title":"Neurology"},{"key":"BFEMMM202215851_CR18","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.15252\/emmm.201505256","volume":"7","author":"S Hwang","year":"2015","unstructured":"Hwang S, Disatnik MH, Mochly\u2010Rosen D (2015) Impaired GAPDH\u2010induced mitophagy contributes to the pathology of Huntington's disease. EMBO Mol Med 7: 1307\u20131326","journal-title":"EMBO Mol Med"},{"key":"BFEMMM202215851_CR19","doi-asserted-by":"crossref","first-page":"103","DOI":"10.3389\/fncel.2019.00103","volume":"13","author":"TA Intihar","year":"2019","unstructured":"Intihar TA, Martinez EA, Gomez\u2010Pastor R (2019) Mitochondrial Dysfunction in Huntington's disease; Interplay between HSF1, p53 and PGC\u20101alpha transcription factors. Front Cell Neurosci 13: 103","journal-title":"Front Cell Neurosci"},{"key":"BFEMMM202215851_CR20","doi-asserted-by":"crossref","first-page":"4573","DOI":"10.1093\/hmg\/ddv188","volume":"24","author":"A Jahani\u2010Asl","year":"2015","unstructured":"Jahani\u2010Asl A, Huang E, Irrcher I, Rashidian J, Ishihara N, Lagace DC, Slack RS, Park DS (2015) CDK5 phosphorylates DRP1 and drives mitochondrial defects in NMDA\u2010induced neuronal death. Hum Mol Genet 24: 4573\u20134583","journal-title":"Hum Mol Genet"},{"key":"BFEMMM202215851_CR21","doi-asserted-by":"crossref","first-page":"11287","DOI":"10.1093\/nar\/gky875","volume":"46","author":"P Kaur","year":"2018","unstructured":"Kaur P, Longley MJ, Pan H, Wang H, Copeland WC (2018) Single\u2010molecule DREEM imaging reveals DNA wrapping around human mitochondrial single\u2010stranded DNA binding protein. Nucleic Acids Res 46: 11287\u201311302","journal-title":"Nucleic Acids Res"},{"key":"BFEMMM202215851_CR22","doi-asserted-by":"crossref","first-page":"2423","DOI":"10.1038\/sj.emboj.7600257","volume":"23","author":"JA Korhonen","year":"2004","unstructured":"Korhonen JA, Pham XH, Pellegrini M, Falkenberg M (2004) Reconstitution of a minimal mtDNA replisome in\u2009vitro. EMBO J 23: 2423\u20132429","journal-title":"EMBO J"},{"key":"BFEMMM202215851_CR23","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1038\/ncb3121","volume":"17","author":"N Kourtis","year":"2015","unstructured":"Kourtis N, Moubarak RS, Aranda\u2010Orgilles B, Lui K, Aydin IT, Trimarchi T, Darvishian F, Salvaggio C, Zhong J, Bhatt K et\u2009al (2015) FBXW7 modulates cellular stress response and metastatic potential through HSF1 post\u2010translational modification. Nat Cell Biol 17: 322\u2013332","journal-title":"Nat Cell Biol"},{"key":"BFEMMM202215851_CR24","doi-asserted-by":"crossref","first-page":"3306","DOI":"10.1172\/JCI57413","volume":"121","author":"J Labbadia","year":"2011","unstructured":"Labbadia J, Cunliffe H, Weiss A, Katsyuba E, Sathasivam K, Seredenina T, Woodman B, Moussaoui S, Frentzel S, Luthi\u2010Carter R et\u2009al (2011) Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington's disease. J Clin Invest 121: 3306\u20133319","journal-title":"J Clin Invest"},{"key":"BFEMMM202215851_CR25","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1038\/nbt.3906","volume":"35","author":"MA Lancaster","year":"2017","unstructured":"Lancaster MA, Corsini NS, Wolfinger S, Gustafson EH, Phillips AW, Burkard TR, Otani T, Livesey FJ, Knoblich JA (2017) Guided self\u2010organization and cortical plate formation in human brain organoids. Nat Biotechnol 35: 659\u2013666","journal-title":"Nat Biotechnol"},{"key":"BFEMMM202215851_CR26","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1038\/nature12517","volume":"501","author":"MA Lancaster","year":"2013","unstructured":"Lancaster MA, Renner M, Martin CA, Wenzel D, Bicknell LS, Hurles ME, Homfray T, Penninger JM, Jackson AP, Knoblich JA (2013) Cerebral organoids model human brain development and microcephaly. Nature 501: 373\u2013379","journal-title":"Nature"},{"key":"BFEMMM202215851_CR27","doi-asserted-by":"crossref","first-page":"1670","DOI":"10.1038\/nprot.2013.106","volume":"8","author":"Y Liu","year":"2013","unstructured":"Liu Y, Liu H, Sauvey C, Yao L, Zarnowska ED, Zhang SC (2013) Directed differentiation of forebrain GABA interneurons from human pluripotent stem cells. Nat Protoc 8: 1670\u20131679","journal-title":"Nat Protoc"},{"key":"BFEMMM202215851_CR28","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1016\/j.stem.2012.01.021","volume":"10","author":"L Ma","year":"2012","unstructured":"Ma L, Hu B, Liu Y, Vermilyea SC, Liu H, Gao L, Sun Y, Zhang X, Zhang SC (2012) Human embryonic stem cell\u2010derived GABA neurons correct locomotion deficits in quinolinic acid\u2010lesioned mice. Cell Stem Cell 10: 455\u2013464","journal-title":"Cell Stem Cell"},{"key":"BFEMMM202215851_CR29","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1016\/j.cmet.2015.08.005","volume":"22","author":"X Ma","year":"2015","unstructured":"Ma X, Xu L, Alberobello AT, Gavrilova O, Bagattin A, Skarulis M, Liu J, Finkel T, Mueller E (2015) Celastrol protects against obesity and metabolic dysfunction through activation of a HSF1\u2010PGC1alpha transcriptional axis. Cell Metab 22: 695\u2013708","journal-title":"Cell Metab"},{"key":"BFEMMM202215851_CR30","first-page":"2313","volume":"127","author":"TD MacVicar","year":"2014","unstructured":"MacVicar TD, Lane JD (2014) Impaired OMA1\u2010dependent cleavage of OPA1 and reduced DRP1 fission activity combine to prevent mitophagy in cells that are dependent on oxidative phosphorylation. J Cell Sci 127: 2313\u20132325","journal-title":"J Cell Sci"},{"key":"BFEMMM202215851_CR31","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1004832","volume":"10","author":"J Miralles Fuste","year":"2014","unstructured":"Miralles Fuste J, Shi Y, Wanrooij S, Zhu X, Jemt E, Persson O, Sabouri N, Gustafsson CM, Falkenberg M (2014) In vivo occupancy of mitochondrial single\u2010stranded DNA binding protein supports the strand displacement mode of DNA replication. PLoS Genet 10: e1004832","journal-title":"PLoS Genet"},{"key":"BFEMMM202215851_CR32","doi-asserted-by":"crossref","first-page":"3788","DOI":"10.1101\/gad.12.24.3788","volume":"12","author":"RI Morimoto","year":"1998","unstructured":"Morimoto RI (1998) Regulation of the heat shock transcriptional response: cross talk between a family of heat shock factors, molecular chaperones, and negative regulators. Genes Dev 12: 3788\u20133796","journal-title":"Genes Dev"},{"key":"BFEMMM202215851_CR33","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1016\/j.celrep.2014.09.056","volume":"9","author":"DW Neef","year":"2014","unstructured":"Neef DW, Jaeger AM, Gomez\u2010Pastor R, Willmund F, Frydman J, Thiele DJ (2014) A direct regulatory interaction between chaperonin TRiC and stress\u2010responsive transcription factor HSF1. Cell Rep 9: 955\u2013966","journal-title":"Cell Rep"},{"key":"BFEMMM202215851_CR34","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1172\/JCI128513","volume":"130","author":"C Piro\u2010Megy","year":"2020","unstructured":"Piro\u2010Megy C, Sarzi E, Tarres\u2010Sole A, Pequignot M, Hensen F, Quiles M, Manes G, Chakraborty A, Senechal A, Bocquet B et\u2009al (2020) Dominant mutations in mtDNA maintenance gene SSBP1 cause optic atrophy and foveopathy. J Clin Invest 130: 143\u2013156","journal-title":"J Clin Invest"},{"key":"BFEMMM202215851_CR35","first-page":"789","volume":"126","author":"X Qi","year":"2013","unstructured":"Qi X, Qvit N, Su YC, Mochly\u2010Rosen D (2013) A novel Drp1 inhibitor diminishes aberrant mitochondrial fission and neurotoxicity. J Cell Sci 126: 789\u2013802","journal-title":"J Cell Sci"},{"key":"BFEMMM202215851_CR36","volume":"6","author":"Z Qi","year":"2011","unstructured":"Qi Z, He J, Su Y, He Q, Liu J, Yu L, Al\u2010Attas O, Hussain T, Ding S, Ji L et\u2009al (2011) Physical exercise regulates p53 activity targeting SCO2 and increases mitochondrial COX biogenesis in cardiac muscle with age. PLoS One 6: e21140","journal-title":"PLoS One"},{"key":"BFEMMM202215851_CR37","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1083\/jcb.201607091","volume":"216","author":"A Qiao","year":"2017","unstructured":"Qiao A, Jin X, Pang J, Moskophidis D, Mivechi NF (2017) The transcriptional regulator of the chaperone response HSF1 controls hepatic bioenergetics and protein homeostasis. J Cell Biol 216: 723\u2013741","journal-title":"J Cell Biol"},{"key":"BFEMMM202215851_CR38","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.bbadis.2011.10.016","volume":"1822","author":"PH Reddy","year":"2012","unstructured":"Reddy PH, Shirendeb UP (2012) Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease. Biochim Biophys Acta 1822: 101\u2013110","journal-title":"Biochim Biophys Acta"},{"key":"BFEMMM202215851_CR39","doi-asserted-by":"crossref","first-page":"5733","DOI":"10.1073\/pnas.85.15.5733","volume":"85","author":"A Reiner","year":"1988","unstructured":"Reiner A, Albin RL, Anderson KD, D'Amato CJ, Penney JB, Young AB (1988) Differential loss of striatal projection neurons in Huntington disease. Proc Natl Acad Sci U S A 85: 5733\u20135737","journal-title":"Proc Natl Acad Sci U S A"},{"key":"BFEMMM202215851_CR40","doi-asserted-by":"crossref","first-page":"931","DOI":"10.1016\/j.bbamcr.2010.04.008","volume":"1803","author":"H Ruhanen","year":"2010","unstructured":"Ruhanen H, Borrie S, Szabadkai G, Tyynismaa H, Jones AW, Kang D, Taanman JW, Yasukawa T (2010) Mitochondrial single\u2010stranded DNA binding protein is required for maintenance of mitochondrial DNA and 7S DNA but is not required for mitochondrial nucleoid organisation. Biochim Biophys Acta 1803: 931\u2013939","journal-title":"Biochim Biophys Acta"},{"key":"BFEMMM202215851_CR41","doi-asserted-by":"crossref","first-page":"6350","DOI":"10.1007\/s12035-021-02556-x","volume":"58","author":"N Sawant","year":"2021","unstructured":"Sawant N, Morton H, Kshirsagar S, Reddy AP, Reddy PH (2021) Mitochondrial abnormalities and synaptic damage in Huntington's disease: a focus on defective mitophagy and mitochondria\u2010targeted therapeutics. Mol Neurobiol 58: 6350\u20136377","journal-title":"Mol Neurobiol"},{"key":"BFEMMM202215851_CR42","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1038\/nrd.2016.245","volume":"16","author":"Y Shi","year":"2017","unstructured":"Shi Y, Inoue H, Wu JC, Yamanaka S (2017) Induced pluripotent stem cell technology: a decade of progress. Nat Rev Drug Discov 16: 115\u2013130","journal-title":"Nat Rev Drug Discov"},{"key":"BFEMMM202215851_CR43","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1093\/hmg\/ddr475","volume":"21","author":"UP Shirendeb","year":"2012","unstructured":"Shirendeb UP, Calkins MJ, Manczak M, Anekonda V, Dufour B, McBride JL, Mao P, Reddy PH (2012) Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease. Hum Mol Genet 21: 406\u2013420","journal-title":"Hum Mol Genet"},{"key":"BFEMMM202215851_CR44","doi-asserted-by":"crossref","first-page":"1438","DOI":"10.1093\/hmg\/ddr024","volume":"20","author":"U Shirendeb","year":"2011","unstructured":"Shirendeb U, Reddy AP, Manczak M, Calkins MJ, Mao P, Tagle DA, Reddy PH (2011) Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage. Hum Mol Genet 20: 1438\u20131455","journal-title":"Hum Mol Genet"},{"key":"BFEMMM202215851_CR45","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1038\/nm.2313","volume":"17","author":"W Song","year":"2011","unstructured":"Song W, Chen J, Petrilli A, Liot G, Klinglmayr E, Zhou Y, Poquiz P, Tjong J, Pouladi MA, Hayden MR et\u2009al (2011) Mutant huntingtin binds the mitochondrial fission GTPase dynamin\u2010related protein\u20101 and increases its enzymatic activity. Nat Med 17: 377\u2013382","journal-title":"Nat Med"},{"key":"BFEMMM202215851_CR46","doi-asserted-by":"crossref","DOI":"10.1126\/sciadv.aaz4452","volume":"6","author":"S Sural","year":"2020","unstructured":"Sural S, Liang CY, Wang FY, Ching TT, Hsu AL (2020) HSB\u20101\/HSF\u20101 pathway modulates histone H4 in mitochondria to control mtDNA transcription and longevity. Sci Adv 6: eaaz4452","journal-title":"Sci Adv"},{"key":"BFEMMM202215851_CR47","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1038\/s41582-020-0389-4","volume":"16","author":"SJ Tabrizi","year":"2020","unstructured":"Tabrizi SJ, Flower MD, Ross CA, Wild EJ (2020) Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities. Nat Rev Neurol 16: 529\u2013546","journal-title":"Nat Rev Neurol"},{"key":"BFEMMM202215851_CR48","doi-asserted-by":"crossref","first-page":"6580","DOI":"10.1038\/ncomms7580","volume":"6","author":"K Tan","year":"2015","unstructured":"Tan K, Fujimoto M, Takii R, Takaki E, Hayashida N, Nakai A (2015) Mitochondrial SSBP1 protects cells from proteotoxic stresses by potentiating stress\u2010induced HSF1 transcriptional activity. Nat Commun 6: 6580","journal-title":"Nat Commun"},{"key":"BFEMMM202215851_CR49","doi-asserted-by":"crossref","first-page":"5164","DOI":"10.1093\/emboj\/cdf528","volume":"21","author":"LJ Yan","year":"2002","unstructured":"Yan LJ, Christians ES, Liu L, Xiao X, Sohal RS, Benjamin IJ (2002) Mouse heat shock transcription factor 1 deficiency alters cardiac redox homeostasis and increases mitochondrial oxidative damage. EMBO J 21: 5164\u20135172","journal-title":"EMBO J"},{"key":"BFEMMM202215851_CR50","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1089\/ars.2005.7.465","volume":"7","author":"LJ Yan","year":"2005","unstructured":"Yan LJ, Rajasekaran NS, Sathyanarayanan S, Benjamin IJ (2005) Mouse HSF1 disruption perturbs redox state and increases mitochondrial oxidative stress in kidney. Antioxid Redox Signal 7: 465\u2013471","journal-title":"Antioxid Redox Signal"},{"key":"BFEMMM202215851_CR51","doi-asserted-by":"crossref","first-page":"1737","DOI":"10.1074\/jbc.M110.179440","volume":"286","author":"YJ Yoon","year":"2011","unstructured":"Yoon YJ, Kim JA, Shin KD, Shin DS, Han YM, Lee YJ, Lee JS, Kwon BM, Han DC (2011) KRIBB11 inhibits HSP70 synthesis through inhibition of heat shock factor 1 function by impairing the recruitment of positive transcription elongation factor b to the hsp70 promoter. J Biol Chem 286: 1737\u20131747","journal-title":"J Biol Chem"},{"key":"BFEMMM202215851_CR52","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.37382","volume":"7","author":"F Yuan","year":"2018","unstructured":"Yuan F, Chen X, Fang KH, Wang Y, Lin M, Xu SB, Huo HQ, Xu M, Ma L, Chen Y et\u2009al (2018) Induction of human somatostatin and parvalbumin neurons by expressing a single transcription factor LIM homeobox 6. eLife 7: e37382","journal-title":"eLife"},{"key":"BFEMMM202215851_CR53","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1016\/j.nbd.2008.03.010","volume":"31","author":"H Zhang","year":"2008","unstructured":"Zhang H, Li Q, Graham RK, Slow E, Hayden MR, Bezprozvanny I (2008) Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease. Neurobiol Dis 31: 80\u201388","journal-title":"Neurobiol Dis"},{"key":"BFEMMM202215851_CR54","doi-asserted-by":"crossref","first-page":"1371","DOI":"10.1038\/s41467-019-09291-x","volume":"10","author":"Y Zhao","year":"2019","unstructured":"Zhao Y, Sun X, Hu D, Prosdocimo DA, Hoppel C, Jain MK, Ramachandran R, Qi X (2019) ATAD3A oligomerization causes neurodegeneration by coupling mitochondrial fragmentation and bioenergetics defects. Nat Commun 10: 1371","journal-title":"Nat Commun"}],"container-title":["EMBO Molecular Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.15252\/emmm.202215851.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.15252\/emmm.202215851","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.15252\/emmm.202215851\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.embopress.org\/doi\/pdf\/10.15252\/emmm.202215851","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.15252\/emmm.202215851.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T06:44:14Z","timestamp":1779086654000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.15252\/emmm.202215851"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6,7]]},"references-count":54,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2022,7,7]]}},"alternative-id":["BFEMMM202215851"],"URL":"https:\/\/doi.org\/10.15252\/emmm.202215851","relation":{},"ISSN":["1757-4676","1757-4684"],"issn-type":[{"value":"1757-4676","type":"print"},{"value":"1757-4684","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,6,7]]},"assertion":[{"value":"8 February 2022","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 May 2022","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 May 2022","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 June 2022","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"EMMM202215851"}}