{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T00:38:57Z","timestamp":1793320737021,"version":"4.5.0"},"reference-count":113,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2020,7,8]],"date-time":"2020-07-08T00:00:00Z","timestamp":1594166400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2020,7,8]],"date-time":"2020-07-08T00:00:00Z","timestamp":1594166400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"funder":[{"name":"Fondecyt","award":["1180186"],"award-info":[{"award-number":["1180186"]}]},{"name":"Fondecyt","award":["1191003"],"award-info":[{"award-number":["1191003"]}]},{"name":"Fondecyt","award":["3150097"],"award-info":[{"award-number":["3150097"]}]},{"name":"Fondecyt","award":["1150069"],"award-info":[{"award-number":["1150069"]}]},{"DOI":"10.13039\/501100018735","name":"Fondap","doi-asserted-by":"crossref","award":["15150012"],"award-info":[{"award-number":["15150012"]}],"id":[{"id":"10.13039\/501100018735","id-type":"DOI","asserted-by":"crossref"}]},{"name":"milenio institute BNI","award":["P09-015-F"],"award-info":[{"award-number":["P09-015-F"]}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["NS092829"],"award-info":[{"award-number":["NS092829"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Acta Neuropathol"],"published-print":{"date-parts":[[2020,11]]},"DOI":"10.1007\/s00401-020-02183-1","type":"journal-article","created":{"date-parts":[[2020,7,8]],"date-time":"2020-07-08T01:33:39Z","timestamp":1594172019000},"page":"737-764","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":62,"title":["Insulin-like growth factor 2 (IGF2) protects against Huntington\u2019s disease through the extracellular disposal of protein aggregates"],"prefix":"10.1007","volume":"140","author":[{"given":"Paula","family":"Garc\u00eda-Huerta","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paulina","family":"Troncoso-Escudero","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Di","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arun","family":"Thiruvalluvan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marisol","family":"Cisternas-Olmedo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel R.","family":"Henr\u00edquez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Plate","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pedro","family":"Chana-Cuevas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristian","family":"Saquel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Thielen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth A.","family":"Longo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brad J.","family":"Geddes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerardo Z.","family":"Lederkremer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neeraj","family":"Sharma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marina","family":"Shenkman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Swati","family":"Naphade","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. Pablo","family":"Sardi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Spichiger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans G.","family":"Richter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felipe A.","family":"Court","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kizito Tshitoko","family":"Tshilenge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa M.","family":"Ellerby","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. Luke","family":"Wiseman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Gonzalez-Billault","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven","family":"Bergink","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rene L.","family":"Vidal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1120-7966","authenticated-orcid":false,"given":"Claudio","family":"Hetz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,7,8]]},"reference":[{"key":"2183_CR1","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1016\/j.molcel.2007.06.011","volume":"27","author":"D Acosta-Alvear","year":"2007","unstructured":"Acosta-Alvear D, Zhou Y, Blais A, Tsikitis M, Lents NH, Arias C et al (2007) XBP1 controls diverse cell type- and condition-specific transcriptional regulatory networks. Mol Cell 27:53\u201366. https:\/\/doi.org\/10.1016\/j.molcel.2007.06.011","journal-title":"Mol Cell"},{"key":"2183_CR2","doi-asserted-by":"publisher","first-page":"4071","DOI":"10.1038\/emboj.2011.293","volume":"30","author":"RC Agis-Balboa","year":"2011","unstructured":"Agis-Balboa RC, Arcos-Diaz D, Wittnam J, Govindarajan N, Blom K, Burkhardt S et al (2011) A hippocampal insulin-growth factor 2 pathway regulates the extinction of fear memories. EMBO J 30:4071\u20134083. https:\/\/doi.org\/10.1038\/emboj.2011.293","journal-title":"EMBO J"},{"key":"2183_CR3","doi-asserted-by":"publisher","first-page":"274","DOI":"10.1016\/j.tins.2011.12.007","volume":"35","author":"CM Alberini","year":"2012","unstructured":"Alberini CM, Chen DY (2012) Memory enhancement: consolidation, reconsolidation and insulin-like growth factor 2. Trends Neurosci 35:274\u2013283. https:\/\/doi.org\/10.1016\/j.tins.2011.12.007","journal-title":"Trends Neurosci"},{"key":"2183_CR4","doi-asserted-by":"publisher","first-page":"25960","DOI":"10.1038\/srep25960","volume":"6","author":"I Allodi","year":"2016","unstructured":"Allodi I, Comley L, Nichterwitz S, Nizzardo M, Simone C, Benitez JA et al (2016) Differential neuronal vulnerability identifies IGF-2 as a protective factor in ALS. Sci Rep 6:25960. https:\/\/doi.org\/10.1038\/srep25960","journal-title":"Sci Rep"},{"key":"2183_CR5","doi-asserted-by":"publisher","first-page":"E5427","DOI":"10.1073\/pnas.1516217112","volume":"112","author":"DT Babcock","year":"2015","unstructured":"Babcock DT, Ganetzky B (2015) Transcellular spreading of huntingtin aggregates in the Drosophila brain. Proc Natl Acad Sci USA 112:E5427\u20135433. https:\/\/doi.org\/10.1073\/pnas.1516217112","journal-title":"Proc Natl Acad Sci USA"},{"key":"2183_CR6","doi-asserted-by":"publisher","first-page":"916","DOI":"10.1126\/science.1141448","volume":"319","author":"WE Balch","year":"2008","unstructured":"Balch WE, Morimoto RI, Dillin A, Kelly JW (2008) Adapting proteostasis for disease intervention. Science 319:916\u2013919. https:\/\/doi.org\/10.1126\/science.1141448","journal-title":"Science"},{"key":"2183_CR7","doi-asserted-by":"publisher","first-page":"15005","DOI":"10.1038\/nrdp.2015.5","volume":"1","author":"GP Bates","year":"2015","unstructured":"Bates GP, Dorsey R, Gusella JF, Hayden MR, Kay C, Leavitt BR et al (2015) Huntington disease. Nat Rev Dis Primers 1:15005. https:\/\/doi.org\/10.1038\/nrdp.2015.5","journal-title":"Nat Rev Dis Primers"},{"key":"2183_CR8","doi-asserted-by":"publisher","first-page":"240","DOI":"10.1159\/000343995","volume":"59","author":"D Bergman","year":"2013","unstructured":"Bergman D, Halje M, Nordin M, Engstrom W (2013) Insulin-like growth factor 2 in development and disease: a mini-review. Gerontology 59:240\u2013249. https:\/\/doi.org\/10.1159\/000343995","journal-title":"Gerontology"},{"key":"2183_CR9","doi-asserted-by":"publisher","first-page":"1449","DOI":"10.1172\/JCI24761","volume":"115","author":"L Bertram","year":"2005","unstructured":"Bertram L, Tanzi RE (2005) The genetic epidemiology of neurodegenerative disease. J Clin Invest 115:1449\u20131457. https:\/\/doi.org\/10.1172\/JCI24761","journal-title":"J Clin Invest"},{"key":"2183_CR10","doi-asserted-by":"publisher","first-page":"3376","DOI":"10.1523\/JNEUROSCI.4248-11.2012","volume":"32","author":"O Bracko","year":"2012","unstructured":"Bracko O, Singer T, Aigner S, Knobloch M, Winner B, Ray J et al (2012) Gene expression profiling of neural stem cells and their neuronal progeny reveals IGF2 as a regulator of adult hippocampal neurogenesis. J Neurosci 32:3376\u20133387. https:\/\/doi.org\/10.1523\/JNEUROSCI.4248-11.2012","journal-title":"J Neurosci"},{"key":"2183_CR11","doi-asserted-by":"publisher","first-page":"1307","DOI":"10.1083\/jcb.110.4.1307","volume":"110","author":"P Caroni","year":"1990","unstructured":"Caroni P, Grandes P (1990) Nerve sprouting in innervated adult skeletal muscle induced by exposure to elevated levels of insulin-like growth factors. J Cell Biol 110:1307\u20131317. https:\/\/doi.org\/10.1083\/jcb.110.4.1307","journal-title":"J Cell Biol"},{"key":"2183_CR12","doi-asserted-by":"publisher","first-page":"R173","DOI":"10.1093\/hmg\/ddw209","volume":"25","author":"SJ Chamberlain","year":"2016","unstructured":"Chamberlain SJ (2016) Disease modelling using human iPSCs. Hum Mol Genet 25:R173\u2013R181. https:\/\/doi.org\/10.1093\/hmg\/ddw209","journal-title":"Hum Mol Genet"},{"key":"2183_CR13","doi-asserted-by":"publisher","first-page":"491","DOI":"10.1038\/nature09667","volume":"469","author":"DY Chen","year":"2011","unstructured":"Chen DY, Stern SA, Garcia-Osta A, Saunier-Rebori B, Pollonini G, Bambah-Mukku D et al (2011) A critical role for IGF-II in memory consolidation and enhancement. Nature 469:491\u2013497. https:\/\/doi.org\/10.1038\/nature09667","journal-title":"Nature"},{"key":"2183_CR14","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1146\/annurev-biochem-061516-045115","volume":"86","author":"F Chiti","year":"2017","unstructured":"Chiti F, Dobson CM (2017) Protein misfolding, amyloid formation, and human disease: a summary of progress over the last decade. Annu Rev Biochem 86:27\u201368. https:\/\/doi.org\/10.1146\/annurev-biochem-061516-045115","journal-title":"Annu Rev Biochem"},{"key":"2183_CR15","doi-asserted-by":"publisher","first-page":"12483","DOI":"10.1073\/pnas.0904239106","volume":"106","author":"F Cicchetti","year":"2009","unstructured":"Cicchetti F, Saporta S, Hauser RA, Parent M, Saint-Pierre M, Sanberg PR et al (2009) Neural transplants in patients with Huntington's disease undergo disease-like neuronal degeneration. Proc Natl Acad Sci USA 106:12483\u201312488. https:\/\/doi.org\/10.1073\/pnas.0904239106","journal-title":"Proc Natl Acad Sci USA"},{"key":"2183_CR16","doi-asserted-by":"publisher","first-page":"849","DOI":"10.1016\/0092-8674(91)90513-x","volume":"64","author":"TM DeChiara","year":"1991","unstructured":"DeChiara TM, Robertson EJ, Efstratiadis A (1991) Parental imprinting of the mouse insulin-like growth factor II gene. Cell 64:849\u2013859. https:\/\/doi.org\/10.1016\/0092-8674(91)90513-x","journal-title":"Cell"},{"key":"2183_CR17","doi-asserted-by":"publisher","first-page":"756","DOI":"10.1038\/nrg1690","volume":"6","author":"NA Di Prospero","year":"2005","unstructured":"Di Prospero NA, Fischbeck KH (2005) Therapeutics development for triplet repeat expansion diseases. Nat Rev Genet 6:756\u2013765. https:\/\/doi.org\/10.1038\/nrg1690","journal-title":"Nat Rev Genet"},{"key":"2183_CR18","doi-asserted-by":"publisher","unstructured":"Dickson DW, Weller RO (2011) Neurodegeneration: the molecular pathology of dementia and movement disorders. https:\/\/doi.org\/10.1002\/9781444341256","DOI":"10.1002\/9781444341256"},{"key":"2183_CR19","doi-asserted-by":"publisher","first-page":"489","DOI":"10.1007\/s00401-017-1694-x","volume":"134","author":"C Duran-Aniotz","year":"2017","unstructured":"Duran-Aniotz C, Cornejo VH, Espinoza S, Ardiles AO, Medinas DB, Salazar C et al (2017) IRE1 signaling exacerbates Alzheimer's disease pathogenesis. Acta Neuropathol 134:489\u2013506. https:\/\/doi.org\/10.1007\/s00401-017-1694-x","journal-title":"Acta Neuropathol"},{"key":"2183_CR20","doi-asserted-by":"publisher","first-page":"482","DOI":"10.1186\/s12864-015-1689-8","volume":"16","author":"LM Eijssen","year":"2015","unstructured":"Eijssen LM, Goelela VS, Kelder T, Adriaens ME, Evelo CT, Radonjic M (2015) A user-friendly workflow for analysis of Illumina gene expression bead array data available at the arrayanalysisorg portal. BMC Genom 16:482. https:\/\/doi.org\/10.1186\/s12864-015-1689-8","journal-title":"BMC Genom"},{"key":"2183_CR21","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1111\/j.1365-2184.1998.tb01196.x","volume":"31","author":"W Engstrom","year":"1998","unstructured":"Engstrom W, Shokrai A, Otte K, Granerus M, Gessbo A, Bierke P et al (1998) Transcriptional regulation and biological significance of the insulin like growth factor II gene. Cell Prolif 31:173\u2013189. https:\/\/doi.org\/10.1111\/j.1365-2184.1998.tb01196.x","journal-title":"Cell Prolif"},{"key":"2183_CR22","doi-asserted-by":"publisher","first-page":"629","DOI":"10.1038\/nature01148","volume":"420","author":"S Etienne-Manneville","year":"2002","unstructured":"Etienne-Manneville S, Hall A (2002) Rho GTPases in cell biology. Nature 420:629\u2013635. https:\/\/doi.org\/10.1038\/nature01148","journal-title":"Nature"},{"key":"2183_CR23","doi-asserted-by":"crossref","first-page":"2933","DOI":"10.1242\/dev.120.10.2933","volume":"120","author":"R Feil","year":"1994","unstructured":"Feil R, Walter J, Allen ND, Reik W (1994) Developmental control of allelic methylation in the imprinted mouse Igf2 and H19 genes. Development 120:2933\u20132943","journal-title":"Development"},{"key":"2183_CR24","doi-asserted-by":"publisher","first-page":"225","DOI":"10.1038\/nrn3209","volume":"13","author":"AM Fernandez","year":"2012","unstructured":"Fernandez AM, Torres-Aleman I (2012) The many faces of insulin-like peptide signalling in the brain. Nat Rev Neurosci 13:225\u2013239. https:\/\/doi.org\/10.1038\/nrn3209","journal-title":"Nat Rev Neurosci"},{"key":"2183_CR25","doi-asserted-by":"publisher","first-page":"8265","DOI":"10.1038\/ncomms9265","volume":"6","author":"SR Ferron","year":"2015","unstructured":"Ferron SR, Radford EJ, Domingo-Muelas A, Kleine I, Ramme A, Gray D et al (2015) Differential genomic imprinting regulates paracrine and autocrine roles of IGF2 in mouse adult neurogenesis. Nat Commun 6:8265. https:\/\/doi.org\/10.1038\/ncomms9265","journal-title":"Nat Commun"},{"key":"2183_CR26","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1016\/j.brainres.2016.02.052","volume":"1649","author":"P Garcia-Huerta","year":"2016","unstructured":"Garcia-Huerta P, Troncoso-Escudero P, Jerez C, Hetz C, Vidal RL (2016) The intersection between growth factors, autophagy and ER stress: a new target to treat neurodegenerative diseases? Brain Res 1649:173\u2013180. https:\/\/doi.org\/10.1016\/j.brainres.2016.02.052","journal-title":"Brain Res"},{"key":"2183_CR27","doi-asserted-by":"publisher","first-page":"1179","DOI":"10.1016\/j.cell.2006.04.026","volume":"125","author":"RK Graham","year":"2006","unstructured":"Graham RK, Deng Y, Slow EJ, Haigh B, Bissada N, Lu G et al (2006) Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell 125:1179\u20131191. https:\/\/doi.org\/10.1016\/j.cell.2006.04.026","journal-title":"Cell"},{"key":"2183_CR28","doi-asserted-by":"publisher","first-page":"444","DOI":"10.1016\/j.nbd.2005.08.007","volume":"21","author":"RK Graham","year":"2006","unstructured":"Graham RK, Slow EJ, Deng Y, Bissada N, Lu G, Pearson J et al (2006) Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models. Neurobiol Dis 21:444\u2013455. https:\/\/doi.org\/10.1016\/j.nbd.2005.08.007","journal-title":"Neurobiol Dis"},{"key":"2183_CR29","doi-asserted-by":"publisher","first-page":"585","DOI":"10.1523\/JNEUROSCI.2730-05.2006","volume":"26","author":"C Hawkes","year":"2006","unstructured":"Hawkes C, Jhamandas JH, Harris KH, Fu W, MacDonald RG, Kar S (2006) Single transmembrane domain insulin-like growth factor-II\/mannose-6-phosphate receptor regulates central cholinergic function by activating a G-protein-sensitive, protein kinase C-dependent pathway. J Neurosci 26:585\u2013596. https:\/\/doi.org\/10.1523\/JNEUROSCI.2730-05.2006","journal-title":"J Neurosci"},{"key":"2183_CR30","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1016\/j.brainresrev.2003.11.002","volume":"44","author":"C Hawkes","year":"2004","unstructured":"Hawkes C, Kar S (2004) The insulin-like growth factor-II\/mannose-6-phosphate receptor: structure, distribution and function in the central nervous system. Brain Res Brain Res Rev 44:117\u2013140. https:\/\/doi.org\/10.1016\/j.brainresrev.2003.11.002","journal-title":"Brain Res Brain Res Rev"},{"key":"2183_CR31","doi-asserted-by":"publisher","first-page":"9730","DOI":"10.1073\/pnas.1002575107","volume":"107","author":"S Henis-Korenblit","year":"2010","unstructured":"Henis-Korenblit S, Zhang P, Hansen M, McCormick M, Lee SJ, Cary M et al (2010) Insulin\/IGF-1 signaling mutants reprogram ER stress response regulators to promote longevity. Proc Natl Acad Sci USA 107:9730\u20139735. https:\/\/doi.org\/10.1073\/pnas.1002575107","journal-title":"Proc Natl Acad Sci USA"},{"key":"2183_CR32","doi-asserted-by":"publisher","first-page":"e53123","DOI":"10.1371\/journal.pone.0053123","volume":"7","author":"DR Henriquez","year":"2012","unstructured":"Henriquez DR, Bodaleo FJ, Montenegro-Venegas C, Gonzalez-Billault C (2012) The light chain 1 subunit of the microtubule-associated protein 1B (MAP1B) is responsible for Tiam1 binding and Rac1 activation in neuronal cells. PLoS ONE 7:e53123. https:\/\/doi.org\/10.1371\/journal.pone.0053123","journal-title":"PLoS ONE"},{"key":"2183_CR33","doi-asserted-by":"publisher","first-page":"89","DOI":"10.1038\/nrm3270","volume":"13","author":"C Hetz","year":"2012","unstructured":"Hetz C (2012) The unfolded protein response: controlling cell fate decisions under ER stress and beyond. Nat Rev Mol Cell Biol 13:89\u2013102. https:\/\/doi.org\/10.1038\/nrm3270","journal-title":"Nat Rev Mol Cell Biol"},{"key":"2183_CR34","doi-asserted-by":"publisher","first-page":"757","DOI":"10.1073\/pnas.0711094105","volume":"105","author":"C Hetz","year":"2008","unstructured":"Hetz C, Lee AH, Gonzalez-Romero D, Thielen P, Castilla J, Soto C et al (2008) Unfolded protein response transcription factor XBP-1 does not influence prion replication or pathogenesis. Proc Natl Acad Sci USA 105:757\u2013762. https:\/\/doi.org\/10.1073\/pnas.0711094105","journal-title":"Proc Natl Acad Sci USA"},{"key":"2183_CR35","doi-asserted-by":"publisher","first-page":"169","DOI":"10.1016\/j.molcel.2017.06.017","volume":"69","author":"C Hetz","year":"2018","unstructured":"Hetz C, Papa FR (2018) The unfolded protein response and cell fate control. Mol Cell 69:169\u2013181. https:\/\/doi.org\/10.1016\/j.molcel.2017.06.017","journal-title":"Mol Cell"},{"key":"2183_CR36","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1038\/nrneurol.2017.99","volume":"13","author":"C Hetz","year":"2017","unstructured":"Hetz C, Saxena S (2017) ER stress and the unfolded protein response in neurodegeneration. Nat Rev Neurol 13:477\u2013491. https:\/\/doi.org\/10.1038\/nrneurol.2017.99","journal-title":"Nat Rev Neurol"},{"key":"2183_CR37","doi-asserted-by":"publisher","first-page":"2294","DOI":"10.1101\/gad.1830709","volume":"23","author":"C Hetz","year":"2009","unstructured":"Hetz C, Thielen P, Matus S, Nassif M, Court F, Kiffin R et al (2009) XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy. Genes Dev 23:2294\u20132306. https:\/\/doi.org\/10.1101\/gad.1830709","journal-title":"Genes Dev"},{"key":"2183_CR38","doi-asserted-by":"publisher","first-page":"2999","DOI":"10.1158\/1078-0432.CCR-09-3233","volume":"16","author":"GS Huang","year":"2010","unstructured":"Huang GS, Brouwer-Visser J, Ramirez MJ, Kim CH, Hebert TM, Lin J et al (2010) Insulin-like growth factor 2 expression modulates Taxol resistance and is a candidate biomarker for reduced disease-free survival in ovarian cancer. Clin Cancer Res 16:2999\u20133010. https:\/\/doi.org\/10.1158\/1078-0432.CCR-09-3233","journal-title":"Clin Cancer Res"},{"key":"2183_CR39","doi-asserted-by":"publisher","first-page":"577","DOI":"10.1007\/s00401-016-1582-9","volume":"132","author":"I Jeon","year":"2016","unstructured":"Jeon I, Cicchetti F, Cisbani G, Lee S, Li E, Bae J et al (2016) Human-to-mouse prion-like propagation of mutant huntingtin protein. Acta Neuropathol 132:577\u2013592. https:\/\/doi.org\/10.1007\/s00401-016-1582-9","journal-title":"Acta Neuropathol"},{"key":"2183_CR40","doi-asserted-by":"publisher","first-page":"1406","DOI":"10.1038\/nm.4001","volume":"21","author":"S Kaushik","year":"2015","unstructured":"Kaushik S, Cuervo AM (2015) Proteostasis and aging. Nat Med 21:1406\u20131415. https:\/\/doi.org\/10.1038\/nm.4001","journal-title":"Nat Med"},{"key":"2183_CR41","doi-asserted-by":"publisher","first-page":"6583","DOI":"10.1113\/JP270655","volume":"594","author":"PJ Kemp","year":"2016","unstructured":"Kemp PJ, Rushton DJ, Yarova PL, Schnell C, Geater C, Hancock JM et al (2016) Improving and accelerating the differentiation and functional maturation of human stem cell-derived neurons: role of extracellular calcium and GABA. J Physiol 594:6583\u20136594. https:\/\/doi.org\/10.1113\/JP270655","journal-title":"J Physiol"},{"key":"2183_CR42","doi-asserted-by":"publisher","first-page":"5948","DOI":"10.2174\/1381612822666160713100235","volume":"22","author":"SM Kessler","year":"2016","unstructured":"Kessler SM, Haybaeck J, Kiemer AK (2016) Insulin-like growth factor 2\u2014the oncogene and its accomplices. Curr Pharm Des 22:5948\u20135961. https:\/\/doi.org\/10.2174\/1381612822666160713100235","journal-title":"Curr Pharm Des"},{"key":"2183_CR43","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1186\/s13029-014-0030-2","volume":"9","author":"BB Khomtchouk","year":"2014","unstructured":"Khomtchouk BB, Van Booven DJ, Wahlestedt C (2014) HeatmapGenerator: high performance RNAseq and microarray visualization software suite to examine differential gene expression levels using an R and C++ hybrid computational pipeline. Source Code Biol Med 9:30. https:\/\/doi.org\/10.1186\/s13029-014-0030-2","journal-title":"Source Code Biol Med"},{"key":"2183_CR44","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/0736-5748(93)90029-d","volume":"11","author":"E Kitraki","year":"1993","unstructured":"Kitraki E, Bozas E, Philippidis H, Stylianopoulou F (1993) Aging-related changes in IGF-II and c-fos gene expression in the rat brain. Int J Dev Neurosci 11:1\u20139. https:\/\/doi.org\/10.1016\/0736-5748(93)90029-d","journal-title":"Int J Dev Neurosci"},{"key":"2183_CR45","doi-asserted-by":"publisher","first-page":"543","DOI":"10.1038\/nature10671","volume":"480","author":"P Koch","year":"2011","unstructured":"Koch P, Breuer P, Peitz M, Jungverdorben J, Kesavan J, Poppe D et al (2011) Excitation-induced ataxin-3 aggregation in neurons from patients with Machado-Joseph disease. Nature 480:543\u2013546. https:\/\/doi.org\/10.1038\/nature10671","journal-title":"Nature"},{"key":"2183_CR46","doi-asserted-by":"publisher","first-page":"7448","DOI":"10.1128\/mcb.23.21.7448-7459.2003","volume":"23","author":"AH Lee","year":"2003","unstructured":"Lee AH, Iwakoshi NN, Glimcher LH (2003) XBP-1 regulates a subset of endoplasmic reticulum resident chaperone genes in the unfolded protein response. Mol Cell Biol 23:7448\u20137459. https:\/\/doi.org\/10.1128\/mcb.23.21.7448-7459.2003","journal-title":"Mol Cell Biol"},{"key":"2183_CR47","doi-asserted-by":"publisher","first-page":"4981","DOI":"10.1021\/jm9002395","volume":"52","author":"R Li","year":"2009","unstructured":"Li R, Pourpak A, Morris SW (2009) Inhibition of the insulin-like growth factor-1 receptor (IGF1R) tyrosine kinase as a novel cancer therapy approach. J Med Chem 52:4981\u20135004. https:\/\/doi.org\/10.1021\/jm9002395","journal-title":"J Med Chem"},{"key":"2183_CR48","doi-asserted-by":"publisher","first-page":"493","DOI":"10.1016\/s0092-8674(00)81369-0","volume":"87","author":"L Mangiarini","year":"1996","unstructured":"Mangiarini L, Sathasivam K, Seller M, Cozens B, Harper A, Hetherington C et al (1996) Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87:493\u2013506. https:\/\/doi.org\/10.1016\/s0092-8674(00)81369-0","journal-title":"Cell"},{"key":"2183_CR49","doi-asserted-by":"publisher","first-page":"615","DOI":"10.1111\/acel.12599","volume":"16","author":"G Martinez","year":"2017","unstructured":"Martinez G, Duran-Aniotz C, Cabral-Miranda F, Vivar JP, Hetz C (2017) Endoplasmic reticulum proteostasis impairment in aging. Aging Cell 16:615\u2013623. https:\/\/doi.org\/10.1111\/acel.12599","journal-title":"Aging Cell"},{"key":"2183_CR50","doi-asserted-by":"publisher","first-page":"104635","DOI":"10.1016\/j.nbd.2019.104635","volume":"134","author":"HS McLoughlin","year":"2020","unstructured":"McLoughlin HS, Moore LR, Paulson HL (2020) Pathogenesis of SCA3 and implications for other polyglutamine diseases. Neurobiol Dis 134:104635. https:\/\/doi.org\/10.1016\/j.nbd.2019.104635","journal-title":"Neurobiol Dis"},{"key":"2183_CR51","doi-asserted-by":"publisher","first-page":"367","DOI":"10.1038\/nature21362","volume":"542","author":"I Melentijevic","year":"2017","unstructured":"Melentijevic I, Toth ML, Arnold ML, Guasp RJ, Harinath G, Nguyen KC et al (2017) C. elegans neurons jettison protein aggregates and mitochondria under neurotoxic stress. Nature 542:367\u2013371. https:\/\/doi.org\/10.1038\/nature21362","journal-title":"Nature"},{"key":"2183_CR52","doi-asserted-by":"publisher","first-page":"e94287","DOI":"10.1371\/journal.pone.0094287","volume":"9","author":"TJ Mellott","year":"2014","unstructured":"Mellott TJ, Pender SM, Burke RM, Langley EA, Blusztajn JK (2014) IGF2 ameliorates amyloidosis, increases cholinergic marker expression and raises BMP9 and neurotrophin levels in the hippocampus of the APPswePS1dE9 Alzheimer's disease model mice. PLoS ONE 9:e94287. https:\/\/doi.org\/10.1371\/journal.pone.0094287","journal-title":"PLoS ONE"},{"key":"2183_CR53","doi-asserted-by":"publisher","first-page":"2311","DOI":"10.1038\/ncomms3311","volume":"4","author":"MA Mikaelsson","year":"2013","unstructured":"Mikaelsson MA, Constancia M, Dent CL, Wilkinson LS, Humby T (2013) Placental programming of anxiety in adulthood revealed by Igf2-null models. Nat Commun 4:2311. https:\/\/doi.org\/10.1038\/ncomms3311","journal-title":"Nat Commun"},{"key":"2183_CR54","doi-asserted-by":"publisher","first-page":"313","DOI":"10.1016\/j.cell.2010.01.028","volume":"140","author":"N Mizushima","year":"2010","unstructured":"Mizushima N, Yoshimori T, Levine B (2010) Methods in mammalian autophagy research. Cell 140:313\u2013326. https:\/\/doi.org\/10.1016\/j.cell.2010.01.028","journal-title":"Cell"},{"key":"2183_CR55","doi-asserted-by":"publisher","first-page":"979","DOI":"10.1007\/s13311-019-00810-8","volume":"16","author":"S Naphade","year":"2019","unstructured":"Naphade S, Tshilenge KT, Ellerby LM (2019) Modeling polyglutamine expansion diseases with induced pluripotent stem cells. Neurotherapeutics 16:979\u2013998. https:\/\/doi.org\/10.1007\/s13311-019-00810-8","journal-title":"Neurotherapeutics"},{"key":"2183_CR56","doi-asserted-by":"publisher","first-page":"11716","DOI":"10.1073\/pnas.89.24.11716","volume":"89","author":"SL Near","year":"1992","unstructured":"Near SL, Whalen LR, Miller JA, Ishii DN (1992) Insulin-like growth factor II stimulates motor nerve regeneration. Proc Natl Acad Sci USA 89:11716\u201311720. https:\/\/doi.org\/10.1073\/pnas.89.24.11716","journal-title":"Proc Natl Acad Sci USA"},{"key":"2183_CR57","doi-asserted-by":"publisher","first-page":"444","DOI":"10.1002\/1521-2254(200011\/12)2:6<444::AID-JGM132>3.0.CO;2-1","volume":"2","author":"CR O'Riordan","year":"2000","unstructured":"O'Riordan CR, Lachapelle AL, Vincent KA, Wadsworth SC (2000) Scaleable chromatographic purification process for recombinant adeno-associated virus (rAAV). J Gene Med 2:444\u2013454. https:\/\/doi.org\/10.1002\/1521-2254(200011\/12)2:6<444:AID-JGM132>3.0.CO;2-1","journal-title":"J Gene Med"},{"key":"2183_CR58","doi-asserted-by":"publisher","first-page":"77","DOI":"10.3389\/fnmol.2014.00077","volume":"7","author":"Z Ortega","year":"2014","unstructured":"Ortega Z, Lucas JJ (2014) Ubiquitin-proteasome system involvement in Huntington's disease. Front Mol Neurosci 7:77. https:\/\/doi.org\/10.3389\/fnmol.2014.00077","journal-title":"Front Mol Neurosci"},{"key":"2183_CR59","doi-asserted-by":"publisher","first-page":"9408","DOI":"10.1523\/JNEUROSCI.2700-12.2013","volume":"33","author":"Y Ouchi","year":"2013","unstructured":"Ouchi Y, Banno Y, Shimizu Y, Ando S, Hasegawa H, Adachi K et al (2013) Reduced adult hippocampal neurogenesis and working memory deficits in the Dgcr8-deficient mouse model of 22q11.2 deletion-associated schizophrenia can be rescued by IGF2. J Neurosci 33:9408\u20139419. https:\/\/doi.org\/10.1523\/JNEUROSCI.2700-12.2013","journal-title":"J Neurosci"},{"key":"2183_CR60","doi-asserted-by":"publisher","unstructured":"Pascual-Lucas M, Viana da Silva S, Di Scala M, Garcia-Barroso C, Gonzalez-Aseguinolaza G, Mulle C, et al (2014) Insulin-like growth factor 2 reverses memory and synaptic deficits in APP transgenic mice. EMBO Mol Med 6:1246\u20131262. https:\/\/doi.org\/10.15252\/emmm.201404228","DOI":"10.15252\/emmm.201404228"},{"key":"2183_CR61","doi-asserted-by":"publisher","first-page":"12382","DOI":"10.1128\/JVI.75.24.12382-12392.2001","volume":"75","author":"MA Passini","year":"2001","unstructured":"Passini MA, Wolfe JH (2001) Widespread gene delivery and structure-specific patterns of expression in the brain after intraventricular injections of neonatal mice with an adeno-associated virus vector. J Virol 75:12382\u201312392. https:\/\/doi.org\/10.1128\/JVI.75.24.12382-12392.2001","journal-title":"J Virol"},{"key":"2183_CR62","doi-asserted-by":"publisher","first-page":"1064","DOI":"10.1038\/nn.3761","volume":"17","author":"E Pecho-Vrieseling","year":"2014","unstructured":"Pecho-Vrieseling E, Rieker C, Fuchs S, Bleckmann D, Esposito MS, Botta P et al (2014) Transneuronal propagation of mutant huntingtin contributes to non-cell autonomous pathology in neurons. Nat Neurosci 17:1064\u20131072. https:\/\/doi.org\/10.1038\/nn.3761","journal-title":"Nat Neurosci"},{"key":"2183_CR63","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.15550","author":"L Plate","year":"2016","unstructured":"Plate L, Cooley CB, Chen JJ, Paxman RJ, Gallagher CM, Madoux F et al (2016) Small molecule proteostasis regulators that reprogram the ER to reduce extracellular protein aggregation. Elife. https:\/\/doi.org\/10.7554\/eLife.15550","journal-title":"Elife"},{"key":"2183_CR64","doi-asserted-by":"publisher","first-page":"2099","DOI":"10.1007\/s00018-012-1156-5","volume":"70","author":"N Porat-Shliom","year":"2013","unstructured":"Porat-Shliom N, Milberg O, Masedunskas A, Weigert R (2013) Multiple roles for the actin cytoskeleton during regulated exocytosis. Cell Mol Life Sci 70:2099\u20132121. https:\/\/doi.org\/10.1007\/s00018-012-1156-5","journal-title":"Cell Mol Life Sci"},{"key":"2183_CR65","doi-asserted-by":"publisher","first-page":"1528","DOI":"10.1093\/hmg\/ddq026","volume":"19","author":"MA Pouladi","year":"2010","unstructured":"Pouladi MA, Xie Y, Skotte NH, Ehrnhoefer DE, Graham RK, Kim JE et al (2010) Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression. Hum Mol Genet 19:1528\u20131538. https:\/\/doi.org\/10.1093\/hmg\/ddq026","journal-title":"Hum Mol Genet"},{"key":"2183_CR66","doi-asserted-by":"publisher","first-page":"1678","DOI":"10.1002\/mrd.20909","volume":"75","author":"DD Pravtcheva","year":"2008","unstructured":"Pravtcheva DD, Wise TL (2008) Igf2r improves the survival and transmission ratio of Igf2 transgenic mice. Mol Reprod Dev 75:1678\u20131687. https:\/\/doi.org\/10.1002\/mrd.20909","journal-title":"Mol Reprod Dev"},{"key":"2183_CR67","doi-asserted-by":"publisher","first-page":"e68357","DOI":"10.1371\/journal.pone.0068357","volume":"8","author":"CC Proenca","year":"2013","unstructured":"Proenca CC, Stoehr N, Bernhard M, Seger S, Genoud C, Roscic A et al (2013) Atg4b-dependent autophagic flux alleviates Huntington's disease progression. PLoS ONE 8:e68357. https:\/\/doi.org\/10.1371\/journal.pone.0068357","journal-title":"PLoS ONE"},{"key":"2183_CR68","doi-asserted-by":"publisher","first-page":"471","DOI":"10.1016\/s0962-8924(01)02153-5","volume":"11","author":"AJ Ridley","year":"2001","unstructured":"Ridley AJ (2001) Rho family proteins: coordinating cell responses. Trends Cell Biol 11:471\u2013477. https:\/\/doi.org\/10.1016\/s0962-8924(01)02153-5","journal-title":"Trends Cell Biol"},{"key":"2183_CR69","doi-asserted-by":"publisher","first-page":"605","DOI":"10.1038\/nmeth.1220","volume":"5","author":"J Riedl","year":"2008","unstructured":"Riedl J, Crevenna AH, Kessenbrock K, Yu JH, Neukirchen D, Bista M et al (2008) Lifeact: a versatile marker to visualize F-actin. Nat Methods 5:605\u2013607. https:\/\/doi.org\/10.1038\/nmeth.1220","journal-title":"Nat Methods"},{"key":"2183_CR70","doi-asserted-by":"publisher","first-page":"247","DOI":"10.3233\/jad-2005-8304","volume":"8","author":"EJ Rivera","year":"2005","unstructured":"Rivera EJ, Goldin A, Fulmer N, Tavares R, Wands JR, de la Monte SM (2005) Insulin and insulin-like growth factor expression and function deteriorate with progression of Alzheimer's disease: link to brain reductions in acetylcholine. J Alzheimers Dis 8:247\u2013268. https:\/\/doi.org\/10.3233\/jad-2005-8304","journal-title":"J Alzheimers Dis"},{"key":"2183_CR71","doi-asserted-by":"publisher","first-page":"1273","DOI":"10.1021\/cb500062n","volume":"9","author":"LM Ryno","year":"2014","unstructured":"Ryno LM, Genereux JC, Naito T, Morimoto RI, Powers ET, Shoulders MD et al (2014) Characterizing the altered cellular proteome induced by the stress-independent activation of heat shock factor 1. ACS Chem Biol 9:1273\u20131283. https:\/\/doi.org\/10.1021\/cb500062n","journal-title":"ACS Chem Biol"},{"key":"2183_CR72","doi-asserted-by":"publisher","first-page":"4136","DOI":"10.1242\/jcs.123000","volume":"126","author":"M Safra","year":"2013","unstructured":"Safra M, Ben-Hamo S, Kenyon C, Henis-Korenblit S (2013) The ire-1 ER stress-response pathway is required for normal secretory-protein metabolism in C. elegans. J Cell Sci 126:4136\u20134146. https:\/\/doi.org\/10.1242\/jcs.123000","journal-title":"J Cell Sci"},{"key":"2183_CR73","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1212\/WNL.0b013e3181e62076","volume":"75","author":"N Saleh","year":"2010","unstructured":"Saleh N, Moutereau S, Azulay JP, Verny C, Simonin C, Tranchant C et al (2010) High insulinlike growth factor I is associated with cognitive decline in Huntington disease. Neurology 75:57\u201363. https:\/\/doi.org\/10.1212\/WNL.0b013e3181e62076","journal-title":"Neurology"},{"key":"2183_CR74","doi-asserted-by":"publisher","first-page":"e0162890","DOI":"10.1371\/journal.pone.0162890","volume":"11","author":"L Salem","year":"2016","unstructured":"Salem L, Saleh N, Desamericq G, Youssov K, Dolbeau G, Cleret L et al (2016) Insulin-like growth factor-1 but not insulin predicts cognitive decline in Huntington's Disease. PLoS ONE 11:e0162890. https:\/\/doi.org\/10.1371\/journal.pone.0162890","journal-title":"PLoS ONE"},{"key":"2183_CR75","doi-asserted-by":"publisher","first-page":"4263","DOI":"10.1111\/j.1742-4658.2008.06562.x","volume":"275","author":"S Sarkar","year":"2008","unstructured":"Sarkar S, Rubinsztein DC (2008) Huntington's disease: degradation of mutant huntingtin by autophagy. FEBS J 275:4263\u20134270. https:\/\/doi.org\/10.1111\/j.1742-4658.2008.06562.x","journal-title":"FEBS J"},{"key":"2183_CR76","doi-asserted-by":"publisher","first-page":"315","DOI":"10.1007\/s00401-015-1462-8","volume":"130","author":"W Scheper","year":"2015","unstructured":"Scheper W, Hoozemans JJ (2015) The unfolded protein response in neurodegenerative diseases: a neuropathological perspective. Acta Neuropathol 130:315\u2013331. https:\/\/doi.org\/10.1007\/s00401-015-1462-8","journal-title":"Acta Neuropathol"},{"key":"2183_CR77","doi-asserted-by":"publisher","first-page":"5688","DOI":"10.1523\/JNEUROSCI.0111-12.2012","volume":"32","author":"MJ Schmeisser","year":"2012","unstructured":"Schmeisser MJ, Baumann B, Johannsen S, Vindedal GF, Jensen V, Hvalby OC et al (2012) IkappaB kinase\/nuclear factor kappaB-dependent insulin-like growth factor 2 (Igf2) expression regulates synapse formation and spine maturation via Igf2 receptor signaling. J Neurosci 32:5688\u20135703. https:\/\/doi.org\/10.1523\/JNEUROSCI.0111-12.2012","journal-title":"J Neurosci"},{"key":"2183_CR78","doi-asserted-by":"publisher","first-page":"1279","DOI":"10.1016\/j.celrep.2013.03.024","volume":"3","author":"MD Shoulders","year":"2013","unstructured":"Shoulders MD, Ryno LM, Genereux JC, Moresco JJ, Tu PG, Wu C et al (2013) Stress-independent activation of XBP1s and\/or ATF6 reveals three functionally diverse ER proteostasis environments. Cell Rep 3:1279\u20131292. https:\/\/doi.org\/10.1016\/j.celrep.2013.03.024","journal-title":"Cell Rep"},{"key":"2183_CR79","doi-asserted-by":"publisher","first-page":"1414","DOI":"10.1097\/WNR.0b013e328330b735","volume":"20","author":"D Silva","year":"2009","unstructured":"Silva D, Dikkes P, Barnes M, Lopez MF (2009) Decreased motoneuron survival in Igf2 null mice after sciatic nerve transection. NeuroReport 20:1414\u20131418. https:\/\/doi.org\/10.1097\/WNR.0b013e328330b735","journal-title":"NeuroReport"},{"key":"2183_CR80","doi-asserted-by":"publisher","first-page":"1555","DOI":"10.1093\/hmg\/ddg169","volume":"12","author":"EJ Slow","year":"2003","unstructured":"Slow EJ, van Raamsdonk J, Rogers D, Coleman SH, Graham RK, Deng Y et al (2003) Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet 12:1555\u20131567. https:\/\/doi.org\/10.1093\/hmg\/ddg169","journal-title":"Hum Mol Genet"},{"key":"2183_CR81","doi-asserted-by":"publisher","first-page":"2113","DOI":"10.1093\/brain\/aww101","volume":"139","author":"HL Smith","year":"2016","unstructured":"Smith HL, Mallucci GR (2016) The unfolded protein response: mechanisms and therapy of neurodegeneration. Brain 139:2113\u20132121. https:\/\/doi.org\/10.1093\/brain\/aww101","journal-title":"Brain"},{"key":"2183_CR82","doi-asserted-by":"publisher","first-page":"e0198820","DOI":"10.1371\/journal.pone.0198820","volume":"13","author":"T Soares Martins","year":"2018","unstructured":"Soares Martins T, Catita J, Martins Rosa I, Abdces O, Henriques AG (2018) Exosome isolation from distinct biofluids using precipitation and column-based approaches. PLoS ONE 13:e0198820. https:\/\/doi.org\/10.1371\/journal.pone.0198820","journal-title":"PLoS ONE"},{"key":"2183_CR83","doi-asserted-by":"publisher","first-page":"1332","DOI":"10.1038\/s41593-018-0235-9","volume":"21","author":"C Soto","year":"2018","unstructured":"Soto C, Pritzkow S (2018) Protein misfolding, aggregation, and conformational strains in neurodegenerative diseases. Nat Neurosci 21:1332\u20131340. https:\/\/doi.org\/10.1038\/s41593-018-0235-9","journal-title":"Nat Neurosci"},{"key":"2183_CR84","doi-asserted-by":"publisher","first-page":"1015","DOI":"10.1523\/JNEUROSCI.2010-17.2017","volume":"38","author":"AB Steinmetz","year":"2018","unstructured":"Steinmetz AB, Stern SA, Kohtz AS, Descalzi G, Alberini CM (2018) Insulin-like growth factor II targets the mTOR pathway to reverse autism-like phenotypes in Mice. J Neurosci 38:1015\u20131029. https:\/\/doi.org\/10.1523\/JNEUROSCI.2010-17.2017","journal-title":"J Neurosci"},{"key":"2183_CR85","doi-asserted-by":"publisher","first-page":"556","DOI":"10.1101\/lm.029348.112","volume":"21","author":"SA Stern","year":"2014","unstructured":"Stern SA, Chen DY, Alberini CM (2014) The effect of insulin and insulin-like growth factors on hippocampus- and amygdala-dependent long-term memory formation. Learn Mem 21:556\u2013563. https:\/\/doi.org\/10.1101\/lm.029348.112","journal-title":"Learn Mem"},{"key":"2183_CR86","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1186\/1742-2094-10-37","volume":"10","author":"HS Suh","year":"2013","unstructured":"Suh HS, Zhao ML, Derico L, Choi N, Lee SC (2013) Insulin-like growth factor 1 and 2 (IGF1, IGF2) expression in human microglia: differential regulation by inflammatory mediators. J Neuroinflammation 10:37. https:\/\/doi.org\/10.1186\/1742-2094-10-37","journal-title":"J Neuroinflammation"},{"key":"2183_CR87","doi-asserted-by":"publisher","first-page":"1435","DOI":"10.1016\/j.cell.2013.05.042","volume":"153","author":"RC Taylor","year":"2013","unstructured":"Taylor RC, Dillin A (2013) XBP-1 is a cell-nonautonomous regulator of stress resistance and longevity. Cell 153:1435\u20131447. https:\/\/doi.org\/10.1016\/j.cell.2013.05.042","journal-title":"Cell"},{"key":"2183_CR88","doi-asserted-by":"crossref","unstructured":"Thiruvalluvan A, P. de Mattos E, Brunsting JF, Bakels R, Serlidaki D, Barazzuol L et al (2020) DNAJB6, a key factor in neuronal sensitivity to amyloidogenesis. Mol Cell (in press)","DOI":"10.1016\/j.molcel.2020.02.022"},{"key":"2183_CR89","doi-asserted-by":"publisher","first-page":"23631","DOI":"10.1074\/jbc.M114.635565","volume":"290","author":"M Torres","year":"2015","unstructured":"Torres M, Medinas DB, Matamala JM, Woehlbier U, Cornejo VH, Solda T et al (2015) The protein-disulfide isomerase ERp57 regulates the steady-state levels of the prion protein. J Biol Chem 290:23631\u201323645. https:\/\/doi.org\/10.1074\/jbc.M114.635565","journal-title":"J Biol Chem"},{"key":"2183_CR90","doi-asserted-by":"publisher","first-page":"9000","DOI":"10.1523\/JNEUROSCI.0118-17.2017","volume":"37","author":"K Trajkovic","year":"2017","unstructured":"Trajkovic K, Jeong H, Krainc D (2017) Mutant Huntingtin is secreted via a late endosomal\/lysosomal unconventional secretory pathway. J Neurosci 37:9000\u20139012. https:\/\/doi.org\/10.1523\/JNEUROSCI.0118-17.2017","journal-title":"J Neurosci"},{"key":"2183_CR91","doi-asserted-by":"publisher","first-page":"108","DOI":"10.1523\/JNEUROSCI.4253-04.2005","volume":"25","author":"BJ Turner","year":"2005","unstructured":"Turner BJ, Atkin JD, Farg MA, Zang DW, Rembach A, Lopes EC et al (2005) Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis. J Neurosci 25:108\u2013117. https:\/\/doi.org\/10.1523\/JNEUROSCI.4253-04.2005","journal-title":"J Neurosci"},{"key":"2183_CR92","doi-asserted-by":"publisher","first-page":"5116","DOI":"10.1073\/pnas.091062498","volume":"98","author":"VG Tusher","year":"2001","unstructured":"Tusher VG, Tibshirani R, Chu G (2001) Significance analysis of microarrays applied to the ionizing radiation response. Proc Natl Acad Sci USA 98:5116\u20135121. https:\/\/doi.org\/10.1073\/pnas.091062498","journal-title":"Proc Natl Acad Sci USA"},{"key":"2183_CR93","doi-asserted-by":"publisher","first-page":"6804","DOI":"10.1073\/pnas.1321845111","volume":"111","author":"P Valdes","year":"2014","unstructured":"Valdes P, Mercado G, Vidal RL, Molina C, Parsons G, Court FA et al (2014) Control of dopaminergic neuron survival by the unfolded protein response transcription factor XBP1. Proc Natl Acad Sci USA 111:6804\u20136809. https:\/\/doi.org\/10.1073\/pnas.1321845111","journal-title":"Proc Natl Acad Sci USA"},{"key":"2183_CR94","doi-asserted-by":"publisher","first-page":"1379","DOI":"10.1093\/hmg\/ddi147","volume":"14","author":"JM Van Raamsdonk","year":"2005","unstructured":"Van Raamsdonk JM, Pearson J, Rogers DA, Bissada N, Vogl AW, Hayden MR et al (2005) Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease. Hum Mol Genet 14:1379\u20131392. https:\/\/doi.org\/10.1093\/hmg\/ddi147","journal-title":"Hum Mol Genet"},{"key":"2183_CR95","doi-asserted-by":"publisher","first-page":"4169","DOI":"10.1523\/JNEUROSCI.0590-05.2005","volume":"25","author":"JM Van Raamsdonk","year":"2005","unstructured":"Van Raamsdonk JM, Pearson J, Slow EJ, Hossain SM, Leavitt BR, Hayden MR (2005) Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease. J Neurosci 25:4169\u20134180. https:\/\/doi.org\/10.1523\/JNEUROSCI.0590-05.2005","journal-title":"J Neurosci"},{"key":"2183_CR96","doi-asserted-by":"publisher","first-page":"1","DOI":"10.2174\/156652411794474419","volume":"11","author":"R Vidal","year":"2011","unstructured":"Vidal R, Caballero B, Couve A, Hetz C (2011) Converging pathways in the occurrence of endoplasmic reticulum (ER) stress in Huntington's disease. Curr Mol Med 11:1\u201312. https:\/\/doi.org\/10.2174\/156652411794474419","journal-title":"Curr Mol Med"},{"key":"2183_CR97","doi-asserted-by":"publisher","first-page":"2245","DOI":"10.1093\/hmg\/dds040","volume":"21","author":"RL Vidal","year":"2012","unstructured":"Vidal RL, Figueroa A, Court FA, Thielen P, Molina C, Wirth C et al (2012) Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy. Hum Mol Genet 21:2245\u20132262. https:\/\/doi.org\/10.1093\/hmg\/dds040","journal-title":"Hum Mol Genet"},{"key":"2183_CR98","doi-asserted-by":"publisher","first-page":"83","DOI":"10.1016\/B978-0-444-52014-2.00004-5","volume":"100","author":"JP Vonsattel","year":"2011","unstructured":"Vonsattel JP, Keller C, Cortes Ramirez EP (2011) Huntington's disease\u2014neuropathology. Handb Clin Neurol 100:83\u2013100. https:\/\/doi.org\/10.1016\/B978-0-444-52014-2.00004-5","journal-title":"Handb Clin Neurol"},{"key":"2183_CR99","doi-asserted-by":"publisher","first-page":"559","DOI":"10.1097\/00005072-198511000-00003","volume":"44","author":"JP Vonsattel","year":"1985","unstructured":"Vonsattel JP, Myers RH, Stevens TJ, Ferrante RJ, Bird ED, Richardson EP Jr (1985) Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurol 44:559\u2013577. https:\/\/doi.org\/10.1097\/00005072-198511000-00003","journal-title":"J Neuropathol Exp Neurol"},{"key":"2183_CR100","doi-asserted-by":"publisher","first-page":"116","DOI":"10.1016\/j.conb.2020.02.003","volume":"61","author":"DM Walsh","year":"2020","unstructured":"Walsh DM, Selkoe DJ (2020) Amyloid beta-protein and beyond: the path forward in Alzheimer's disease. Curr Opin Neurobiol 61:116\u2013124. https:\/\/doi.org\/10.1016\/j.conb.2020.02.003","journal-title":"Curr Opin Neurobiol"},{"key":"2183_CR101","doi-asserted-by":"publisher","first-page":"520","DOI":"10.1210\/endo.140.1.6463","volume":"140","author":"HJ Walter","year":"1999","unstructured":"Walter HJ, Berry M, Hill DJ, Cwyfan-Hughes S, Holly JM, Logan A (1999) Distinct sites of insulin-like growth factor (IGF)-II expression and localization in lesioned rat brain: possible roles of IGF binding proteins (IGFBPs) in the mediation of IGF-II activity. Endocrinology 140:520\u2013532. https:\/\/doi.org\/10.1210\/endo.140.1.6463","journal-title":"Endocrinology"},{"key":"2183_CR102","doi-asserted-by":"publisher","first-page":"1081","DOI":"10.1126\/science.1209038","volume":"334","author":"P Walter","year":"2011","unstructured":"Walter P, Ron D (2011) The unfolded protein response: from stress pathway to homeostatic regulation. Science 334:1081\u20131086. https:\/\/doi.org\/10.1126\/science.1209038","journal-title":"Science"},{"key":"2183_CR103","doi-asserted-by":"publisher","first-page":"326","DOI":"10.1038\/nature17041","volume":"529","author":"M Wang","year":"2016","unstructured":"Wang M, Kaufman RJ (2016) Protein misfolding in the endoplasmic reticulum as a conduit to human disease. Nature 529:326\u2013335. https:\/\/doi.org\/10.1038\/nature17041","journal-title":"Nature"},{"key":"2183_CR104","doi-asserted-by":"publisher","first-page":"1979","DOI":"10.1172\/JCI80743","volume":"125","author":"EJ Wild","year":"2015","unstructured":"Wild EJ, Boggio R, Langbehn D, Robertson N, Haider S, Miller JR et al (2015) Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients. J Clin Invest 125:1979\u20131986. https:\/\/doi.org\/10.1172\/JCI80743","journal-title":"J Clin Invest"},{"key":"2183_CR105","doi-asserted-by":"publisher","first-page":"194","DOI":"10.1002\/(SICI)1098-2795(199710)48:2<194::AID-MRD7>3.0.CO;2-N","volume":"48","author":"TL Wise","year":"1997","unstructured":"Wise TL, Pravatcheva DD (1997) Perinatal lethality in H19 enhancers-Igf2 transgenic mice. Mol Reprod Dev 48:194\u2013207. https:\/\/doi.org\/10.1002\/(SICI)1098-2795(199710)48:2<194:AID-MRD7>3.0.CO;2-N","journal-title":"Mol Reprod Dev"},{"key":"2183_CR106","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1016\/j.nbd.2014.12.030","volume":"76","author":"BKY Wong","year":"2015","unstructured":"Wong BKY, Ehrnhoefer DE, Graham RK, Martin DDO, Ladha S, Uribe V et al (2015) Partial rescue of some features of Huntington Disease in the genetic absence of caspase-6 in YAC128 mice. Neurobiol Dis 76:24\u201336. https:\/\/doi.org\/10.1016\/j.nbd.2014.12.030","journal-title":"Neurobiol Dis"},{"key":"2183_CR107","doi-asserted-by":"publisher","first-page":"805","DOI":"10.1038\/nn.2575","volume":"13","author":"E Wong","year":"2010","unstructured":"Wong E, Cuervo AM (2010) Autophagy gone awry in neurodegenerative diseases. Nat Neurosci 13:805\u2013811. https:\/\/doi.org\/10.1038\/nn.2575","journal-title":"Nat Neurosci"},{"key":"2183_CR108","doi-asserted-by":"publisher","unstructured":"Zanella ER, Galimi F, Sassi F, Migliardi G, Cottino F, Leto SM et al (2015) IGF2 is an actionable target that identifies a distinct subpopulation of colorectal cancer patients with marginal response to anti-EGFR therapies. Sci Transl Med 7:272ra212. https:\/\/doi.org\/10.1126\/scitranslmed.3010445","DOI":"10.1126\/scitranslmed.3010445"},{"key":"2183_CR109","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1007\/s10571-016-0350-7","volume":"36","author":"X Zhang","year":"2016","unstructured":"Zhang X, Abels ER, Redzic JS, Margulis J, Finkbeiner S, Breakefield XO (2016) Potential transfer of polyglutamine and CAG-repeat RNA in extracellular vesicles in Huntington's disease: background and evaluation in cell culture. Cell Mol Neurobiol 36:459\u2013470. https:\/\/doi.org\/10.1007\/s10571-016-0350-7","journal-title":"Cell Mol Neurobiol"},{"key":"2183_CR110","doi-asserted-by":"publisher","first-page":"8317","DOI":"10.1523\/JNEUROSCI.0806-16.2016","volume":"36","author":"T Zhao","year":"2016","unstructured":"Zhao T, Hong Y, Li S, Li XJ (2016) Compartment-dependent degradation of mutant Huntingtin accounts for its preferential accumulation in neuronal processes. J Neurosci 36:8317\u20138328. https:\/\/doi.org\/10.1523\/JNEUROSCI.0806-16.2016","journal-title":"J Neurosci"},{"key":"2183_CR111","doi-asserted-by":"publisher","first-page":"816","DOI":"10.1016\/j.stemcr.2019.02.011","volume":"12","author":"AN Ziegler","year":"2019","unstructured":"Ziegler AN, Feng Q, Chidambaram S, Testai JM, Kumari E, Rothbard DE et al (2019) Insulin-like growth factor II: an essential adult stem cell niche constituent in brain and intestine. Stem Cell Reports 12:816\u2013830. https:\/\/doi.org\/10.1016\/j.stemcr.2019.02.011","journal-title":"Stem Cell Reports"},{"key":"2183_CR112","doi-asserted-by":"publisher","first-page":"905","DOI":"10.1152\/physrev.00041.2009","volume":"90","author":"C Zuccato","year":"2010","unstructured":"Zuccato C, Valenza M, Cattaneo E (2010) Molecular mechanisms and potential therapeutical targets in Huntington's disease. Physiol Rev 90:905\u2013981. https:\/\/doi.org\/10.1152\/physrev.00041.2009","journal-title":"Physiol Rev"},{"key":"2183_CR113","doi-asserted-by":"publisher","first-page":"558","DOI":"10.1016\/j.bbrc.2012.03.033","volume":"420","author":"A Zuleta","year":"2012","unstructured":"Zuleta A, Vidal RL, Armentano D, Parsons G, Hetz C (2012) AAV-mediated delivery of the transcription factor XBP1s into the striatum reduces mutant Huntingtin aggregation in a mouse model of Huntington's disease. Biochem Biophys Res Commun 420:558\u2013563. https:\/\/doi.org\/10.1016\/j.bbrc.2012.03.033","journal-title":"Biochem Biophys Res Commun"}],"container-title":["Acta Neuropathologica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-020-02183-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00401-020-02183-1\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-020-02183-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,7,7]],"date-time":"2021-07-07T19:58:43Z","timestamp":1625687923000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00401-020-02183-1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,7,8]]},"references-count":113,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2020,11]]}},"alternative-id":["2183"],"URL":"https:\/\/doi.org\/10.1007\/s00401-020-02183-1","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/2020.05.28.119164","asserted-by":"object"}]},"ISSN":["0001-6322","1432-0533"],"issn-type":[{"value":"0001-6322","type":"print"},{"value":"1432-0533","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,7,8]]},"assertion":[{"value":"5 March 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 June 2020","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 June 2020","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 July 2020","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"The authors declare no conflict of interest in this study. AAV-IGF2 for gene therapy and its use in misfolded protein diseases as Huntington\u2019s disease (Chile no. 201603282 and PCT. provisional application no. PCT\/CL2017\/000040).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"MTA may be required for access to AAV-IGF2 and some vectors. All data are available upon request from C.H.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Data and materials availability"}}]}}