{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,3]],"date-time":"2026-11-03T13:52:33Z","timestamp":1793713953908,"version":"4.6.1"},"reference-count":40,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2012,5,13]],"date-time":"2012-05-13T00:00:00Z","timestamp":1336867200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Mol Neurobiol"],"published-print":{"date-parts":[[2012,6]]},"DOI":"10.1007\/s12035-012-8274-9","type":"journal-article","created":{"date-parts":[[2012,5,12]],"date-time":"2012-05-12T07:23:28Z","timestamp":1336807408000},"page":"550-563","source":"Crossref","is-referenced-by-count":38,"title":["Deregulation of BRCA1 Leads to Impaired Spatiotemporal Dynamics of \u03b3-H2AX and DNA Damage Responses in Huntington\u2019s Disease"],"prefix":"10.1007","volume":"45","author":[{"given":"Gye Sun","family":"Jeon","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ki Yoon","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu Jin","family":"Hwang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min-Kyung","family":"Jung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sungkwan","family":"An","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mutsuko","family":"Ouchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toru","family":"Ouchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil","family":"Kowall","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junghee","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hoon","family":"Ryu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2012,5,13]]},"reference":[{"key":"8274_CR1","doi-asserted-by":"crossref","first-page":"3","DOI":"10.2174\/1874609811003010003","volume":"3","author":"F Copped\u00e8","year":"2010","unstructured":"Copped\u00e8 F, Migliore L (2010) DNA repair in premature aging disorders and neurodegeneration. Curr Aging Sci 3:3\u201319","journal-title":"Curr Aging Sci"},{"key":"8274_CR2","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1038\/nature05778","volume":"447","author":"IV Kovtun","year":"2007","unstructured":"Kovtun IV, Liu Y, Bjoras M, Klungland A, Wilson SH, McMurray CT (2007) OGG1 initiates age-dependent CAG trinucleotide expansion in somatic cells. Nature 447:447\u2013452","journal-title":"Nature"},{"key":"8274_CR3","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1016\/S0092-8674(00)80503-6","volume":"90","author":"R Scully","year":"1997","unstructured":"Scully R, Chen J, Ochs RL, Keegan K, Hoekstra M, Feunteun J, Livingston DM (1997) Dynamic changes of BRCA1 subnuclear location and phosphorylation state are initiated by DNA damage. Cell 90:425\u2013435","journal-title":"Cell"},{"issue":"suppl 1","key":"8274_CR4","doi-asserted-by":"crossref","first-page":"R113","DOI":"10.1093\/hmg\/ddg082","volume":"12","author":"CX Deng","year":"2003","unstructured":"Deng CX, Wang RH (2003) Roles of BRCA1 in DNA damage repair: a link between development and cancer. Hum Mol Genet 12(suppl 1):R113\u2013R123","journal-title":"Hum Mol Genet"},{"key":"8274_CR5","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1038\/nrc1435","volume":"4","author":"M Ljungman","year":"2004","unstructured":"Ljungman M, Lane DP (2004) Transcription\u2014guarding the genome by sensing DNA damage. Nat Rev Cancer 4:727\u2013737","journal-title":"Nat Rev Cancer"},{"key":"8274_CR6","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1016\/S0955-0674(03)00042-5","volume":"15","author":"LM Starita","year":"2003","unstructured":"Starita LM, Parvin JD (2003) The multiple nuclear functions of BRCA1: transcription, ubiquitination and DNA repair. Curr Opin Cell Biol 15:345\u2013350","journal-title":"Curr Opin Cell Biol"},{"key":"8274_CR7","doi-asserted-by":"crossref","first-page":"3591","DOI":"10.1242\/jcs.114.20.3591","volume":"114","author":"AR Venkitaraman","year":"2001","unstructured":"Venkitaraman AR (2001) Functions of BRCA1 and BRCA2 in the biological response to DNA damage. J Cell Sci 114:3591\u20133598","journal-title":"J Cell Sci"},{"key":"8274_CR8","first-page":"4074","volume":"56","author":"Y Chen","year":"1996","unstructured":"Chen Y, Farmer AA, Chen CF, Jones DC, Chen PL, Lee WH (1996) BRCA1 is a 220-kDa nuclear phosphoprotein that is expressed and phosphorylated in a cell cycle-dependent manner. Cancer Res 56:4074","journal-title":"Cancer Res"},{"key":"8274_CR9","doi-asserted-by":"crossref","first-page":"7138","DOI":"10.1073\/pnas.94.14.7138","volume":"94","author":"H Ruffner","year":"1997","unstructured":"Ruffner H, Verma IM (1997) BRCA1 is a cell cycle-regulated nuclear phosphoprotein. Proc Natl Acad Sci USA 94:7138\u20137143","journal-title":"Proc Natl Acad Sci USA"},{"key":"8274_CR10","doi-asserted-by":"crossref","first-page":"1162","DOI":"10.1126\/science.286.5442.1162","volume":"286","author":"D Cortez","year":"1999","unstructured":"Cortez D, Wang Y, Qin J, Elledge SJ (1999) Requirement of ATM-dependent phosphorylation of brca1 in the DNA damage response to double-strand breaks. Science 286:1162\u20131166","journal-title":"Science"},{"key":"8274_CR11","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1038\/35004614","volume":"404","author":"JS Lee","year":"2000","unstructured":"Lee JS, Collins KM, Brown AL, Lee CH, Chung JH (2000) hCds1-mediated phosphorylation of BRCA1 regulates the DNA damage response. Nature 404:201\u2013204","journal-title":"Nature"},{"key":"8274_CR12","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1016\/S1097-2765(00)80238-5","volume":"4","author":"R Scully","year":"1999","unstructured":"Scully R, Ganesan S, Vlasakova K, Chen J, Socolovsky M, Livingston DM (1999) Genetic analysis of BRCA1 function in a defined tumor cell line. Mol Cell 4:1093\u20131099","journal-title":"Mol Cell"},{"key":"8274_CR13","doi-asserted-by":"crossref","first-page":"528","DOI":"10.1038\/sj.onc.1206208","volume":"22","author":"R Bachelier","year":"2003","unstructured":"Bachelier R, Xu X, Wang X, Li W, Naramura M, Gu H, Deng CX (2003) Normal lymphocyte development and thymic lymphoma formation in Brca1 exon-11-deficient mice. Oncogene 22:528\u2013537","journal-title":"Oncogene"},{"key":"8274_CR14","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1101\/gad.1050003","volume":"17","author":"L Cao","year":"2003","unstructured":"Cao L, Li W, Kim S, Brodie SG, Deng CX (2003) Senescence, aging, and malignant transformation mediated by p53 in mice lacking the Brca1 full-length isoform. Genes Dev 17:201\u2013213","journal-title":"Genes Dev"},{"key":"8274_CR15","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1038\/ng0296-191","volume":"12","author":"LC Gowen","year":"1996","unstructured":"Gowen LC, Johnson BL, Latour AM, Sulik KK, Koller BH (1996) Brca1 deficiency results in early embryonic lethality characterized by neuroepithelial abnormalities. Nat Genet 12:191\u2013194","journal-title":"Nat Genet"},{"key":"8274_CR16","doi-asserted-by":"crossref","first-page":"1009","DOI":"10.1016\/S0092-8674(00)81302-1","volume":"85","author":"R Hakem","year":"1996","unstructured":"Hakem R, de la Pompa JL, Sirard C, Mo R, Woo M, Hakem A, Wakeham A, Potter J, Reitmair A, Bilia F et al (1996) The tumor suppressor gene Brca1 is required for embryonic cellular proliferation in the mouse. Cell 85:1009\u20131023","journal-title":"Cell"},{"key":"8274_CR17","doi-asserted-by":"crossref","first-page":"2544","DOI":"10.1038\/sj.onc.1204363","volume":"20","author":"P Hohenstein","year":"2001","unstructured":"Hohenstein P, Kielman MF, Breukel C, Bennett LM, Wiseman R, Krimpenfort P, Cornelisse C, van Ommen GJ, Devilee P, Fodde R (2001) A targeted mouse Brca1 mutation removing the last BRCT repeat results in apoptosis and embryonic lethality at the headfold stage. Oncogene 20:2544\u20132550","journal-title":"Oncogene"},{"key":"8274_CR18","doi-asserted-by":"crossref","first-page":"1835","DOI":"10.1101\/gad.10.14.1835","volume":"10","author":"CY Liu","year":"1996","unstructured":"Liu CY, Flesken-Nikitin A, Li S, Zeng Y, Lee WH (1996) Inactivation of the mouse Brca1 gene leads to failure in the morphogenesis of the egg cylinder in early postimplantation development. Genes Dev 10:1835\u20131843","journal-title":"Genes Dev"},{"key":"8274_CR19","doi-asserted-by":"crossref","first-page":"1226","DOI":"10.1101\/gad.11.10.1226","volume":"11","author":"T Ludwig","year":"1997","unstructured":"Ludwig T, Chapman DL, Papaioannou VE, Efstratiadis A (1997) Targeted mutations of breast cancer susceptibility gene homologs in mice: lethal phenotypes of Brca1, Brca2, Brca1\/Brca2, Brca1\/p53, and Brca2\/p53 nullizygous embryos. Genes Dev 11:1226\u20131241","journal-title":"Genes Dev"},{"key":"8274_CR20","doi-asserted-by":"crossref","first-page":"1188","DOI":"10.1101\/gad.879201","volume":"15","author":"T Ludwig","year":"2001","unstructured":"Ludwig T, Fisher P, Ganesan S, Efstratiadis A (2001) Tumorigenesis in mice carrying a truncating Brca1 mutation. Genes Dev 15:1188\u20131893","journal-title":"Genes Dev"},{"key":"8274_CR21","doi-asserted-by":"crossref","first-page":"3115","DOI":"10.1038\/sj.onc.1202243","volume":"17","author":"SX Shen","year":"1998","unstructured":"Shen SX, Weaver Z, Xu X, Li C, Weinstein M, Chen L, Guan XY, Ried T, Deng CX (1998) A targeted disruption of the murine Brca1 gene causes gamma-irradiation hypersensitivity and genetic instability. Oncogene 17:3115\u20133124","journal-title":"Oncogene"},{"key":"8274_CR22","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1016\/S1097-2765(00)80466-9","volume":"3","author":"X Xu","year":"1999","unstructured":"Xu X, Weaver Z, Linke SP, Li C, Gotay J, Wang XW, Harris CC, Ried T, Deng CX (1999) Centrosome amplification and a defective G2-M cell cycle checkpoint induce genetic instability in BRCA1 exon 11 isoform-deficient cells. Mol Cell 3:389\u2013395","journal-title":"Mol Cell"},{"key":"8274_CR23","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1038\/8743","volume":"22","author":"X Xu","year":"1999","unstructured":"Xu X, Wagner KU, Larson D, Weaver Z, Li C, Ried T, Hennighausen L, Wynshaw-Boris A, Deng CX (1999) Conditional mutation of Brca1 in mammary epithelial cells results in blunted ductal morphogenesis and tumour formation. Nat Genet 22:37\u201343","journal-title":"Nat Genet"},{"key":"8274_CR24","doi-asserted-by":"crossref","first-page":"266","DOI":"10.1038\/90108","volume":"28","author":"X Xu","year":"2001","unstructured":"Xu X, Qiao W, Linke SP, Cao L, Li WM, Furth PA, Harris CC, Deng CX (2001) Genetic interactions between tumor suppressors Brca1 and p53 in apoptosis, cell cycle and tumorigenesis. Nat Genet 28:266\u2013271","journal-title":"Nat Genet"},{"key":"8274_CR25","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1016\/S0959-437X(02)00282-4","volume":"12","author":"C Redon","year":"2002","unstructured":"Redon C, Pilch D, Rogakou E, Sedelnikova O, Newrock K, Bonner W (2002) Histone H2A variants H2AX and H2AZ. Curr Opin Genet Dev 12:162\u2013169","journal-title":"Curr Opin Genet Dev"},{"key":"8274_CR26","doi-asserted-by":"crossref","first-page":"886","DOI":"10.1016\/S0960-9822(00)00610-2","volume":"10","author":"TT Paull","year":"2000","unstructured":"Paull TT, Rogakou EP, Yamazaki V, Kirchgessner CU, Gellert M, Bonner WM (2000) A critical role for histone H2AX in recruitment of repair factors to nuclear foci after DNA damage. Curr Biol 10:886\u2013895","journal-title":"Curr Biol"},{"key":"8274_CR27","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1126\/science.1069398","volume":"296","author":"A Celeste","year":"2002","unstructured":"Celeste A, PetersenS RPJ, Fernandez-Capetillo O, Chen HT, Sedelnikova OA, Reina-San-Martin B, Coppola V, Meffre E, Difilippantonio MJ et al (2002) Genomic instability in mice lacking histone H2AX. Science 296:922\u2013927","journal-title":"Science"},{"key":"8274_CR28","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1016\/j.cell.2008.04.021","volume":"133","author":"M Stucki","year":"2008","unstructured":"Stucki M, Clapperton JA, Mohammad D, Yaffe MB, Smerdon SJ, Jackson SP (2008) MDC1 directly binds phosphorylated histone H2AX to regulate cellular responses to DNA double-strand breaks. Cell 133:549","journal-title":"Cell"},{"key":"8274_CR29","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1038\/nature01125","volume":"420","author":"H Tauchi","year":"2002","unstructured":"Tauchi H, Kobayashi J, Morishima K, van Gent DC, Shiraishi T, Verkaik NS, van Heems D, Ito E, Nakamura A, Sonoda E et al (2002) Nbs1 is essential for DNA repair by homologous recombination in higher vertebrate cells. Nature 420:93\u201398","journal-title":"Nature"},{"key":"8274_CR30","doi-asserted-by":"crossref","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, Lawton M, Trottier Y, Lehrach H, Davies SW et al (1996) Exon1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87:493\u2013506","journal-title":"Cell"},{"key":"8274_CR31","doi-asserted-by":"crossref","first-page":"2301","DOI":"10.1093\/hmg\/ddg242","volume":"12","author":"P Giuliano","year":"2003","unstructured":"Giuliano P, De Cristofaro T, Affaitati A, Pizzulo GM, Feliciello A, Crscuolo C, De Michele G, Filla A, Awedimento EV, Varrone S (2003) DNA damage induced by polyglutamine-expanded proteins. Hum Mol Genet 12:2301\u20132309","journal-title":"Hum Mol Genet"},{"key":"8274_CR32","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1002\/jnr.21881","volume":"87","author":"J Illuzzi","year":"2009","unstructured":"Illuzzi J, Yerkes S, Parekh-Olmedo H, Kmiec EB (2009) DNA breakage and induction of DNA damage response proteins precede the appearance of visible mutant huntingtin aggregates. J Neurosci Res 87:733\u2013747","journal-title":"J Neurosci Res"},{"key":"8274_CR33","first-page":"3299","volume":"60","author":"M Gatei","year":"2000","unstructured":"Gatei M, Scott SP, Filippovitch I, Soronika N, Lavin MF, Weber B, Khanna KK (2000) Role for ATM in DNA damage-induced phosphorylation of BRCA1. Cancer Res 60:3299\u20133304","journal-title":"Cancer Res"},{"key":"8274_CR34","doi-asserted-by":"crossref","first-page":"17276","DOI":"10.1074\/jbc.M011681200","volume":"276","author":"M Gatei","year":"2001","unstructured":"Gatei M, Zhou BB, Hobson K, Scott S, Young D, Khanna KK (2001) Ataxia telangiectasia mutated (ATM) kinase and ATM and Rad3 related kinase mediate phosphorylation of Brca1 at distinct and overlapping sites. In vivo assessment using phospho-specific antibodies. J Biol Chem 276:17276\u201317280","journal-title":"J Biol Chem"},{"key":"8274_CR35","doi-asserted-by":"crossref","first-page":"2860","DOI":"10.1093\/emboj\/cdg274","volume":"22","author":"N Foray","year":"2003","unstructured":"Foray N, Marot D, Gabriel A, Randrianarison V, Carr AM, Perricaudet M, Ashworth A, Jeggo P (2003) A subset of ATM- and ATR-dependent phosphorylation events requires the BRCA1 protein. EMBO J 22:2860\u20132871","journal-title":"EMBO J"},{"key":"8274_CR36","doi-asserted-by":"crossref","first-page":"19643","DOI":"10.1074\/jbc.M311780200","volume":"279","author":"M Ouchi","year":"2004","unstructured":"Ouchi M, Fujiuchi N, Sasai K, Katayama H, Minamishima YA, Ongusaha PP, Deng C, Sen S, Lee SW, Ouchi T (2004) BRCA1 phosphorylation by Aurora-A in the regulation of G2 to M transition. J Biol Chem 279:19643\u201319648","journal-title":"J Biol Chem"},{"key":"8274_CR37","doi-asserted-by":"crossref","first-page":"2799","DOI":"10.1093\/hmg\/9.19.2799","volume":"9","author":"F Trettel","year":"2000","unstructured":"Trettel F, Rigamonti D, Hilditch-Maguire P, Wheeler VC, Sharp AH, Persichetti F, Cattaneo E, MacDonald ME (2000) Dominant phenotypes produced by the HD mutation in STHdh (Q111) striatal cells. Hum Mol Genet 9:2799\u20132809","journal-title":"Hum Mol Genet"},{"key":"8274_CR38","doi-asserted-by":"crossref","first-page":"3597","DOI":"10.1523\/JNEUROSCI.23-09-03597.2003","volume":"23","author":"H Ryu","year":"2003","unstructured":"Ryu H, Lee J, Zaman K, Kubilis J, Ferrante RJ, Ross BD, Neve R, Ratan RR (2003) Sp1 and Sp3 are oxidative stress-inducible, anti-death transcription factors in cortical neurons. J Neurosci 23:3597\u20133606","journal-title":"J Neurosci"},{"key":"8274_CR39","doi-asserted-by":"crossref","first-page":"19176","DOI":"10.1073\/pnas.0606373103","volume":"103","author":"H Ryu","year":"2006","unstructured":"Ryu H, Lee J, Hagerty SW, Soh BY, MacAlpin SE, Cormier KA, Smith KM, Ferrante RJ (2006) ESET\/SETDB1 gene expression and histone H3 (K9) trimethylation in Huntington's disease. Proc Natl Acad Sci USA 103:19176\u201319181","journal-title":"Proc Natl Acad Sci USA"},{"key":"8274_CR40","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1038\/labinvest.2010.203","volume":"91","author":"H Ryu","year":"2011","unstructured":"Ryu H, Jeon GS, Cashman NR, Kowall NW, Lee J (2011) Differential expression of c-Ret in motor neurons versus non-neuronal cells is linked to the pathogenesis of ALS. Lab Invest 91:342\u2013352","journal-title":"Lab Invest"}],"container-title":["Molecular Neurobiology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s12035-012-8274-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s12035-012-8274-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s12035-012-8274-9","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,1,15]],"date-time":"2022-01-15T14:01:31Z","timestamp":1642255291000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s12035-012-8274-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,5,13]]},"references-count":40,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2012,6]]}},"alternative-id":["8274"],"URL":"https:\/\/doi.org\/10.1007\/s12035-012-8274-9","relation":{},"ISSN":["0893-7648","1559-1182"],"issn-type":[{"value":"0893-7648","type":"print"},{"value":"1559-1182","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,5,13]]}}}