{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T02:53:34Z","timestamp":1790823214626,"version":"4.1.0"},"reference-count":222,"publisher":"SAGE Publications","issue":"1","license":[{"start":{"date-parts":[[2021,2,9]],"date-time":"2021-02-09T00:00:00Z","timestamp":1612828800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Huntington's Disease"],"published-print":{"date-parts":[[2021,2,9]]},"abstract":"<jats:p>DNA mismatch repair (MMR) is a highly conserved genome stabilizing pathway that corrects DNA replication errors, limits chromosomal rearrangements, and mediates the cellular response to many types of DNA damage. Counterintuitively, MMR is also involved in the generation of mutations, as evidenced by its role in causing somatic triplet repeat expansion in Huntington\u2019s disease (HD) and other neurodegenerative disorders. In this review, we discuss the current state of mechanistic knowledge of MMR and review the roles of key enzymes in this pathway. We also present the evidence for mutagenic function of MMR in CAG repeat expansion and consider mechanistic hypotheses that have been proposed. Understanding the role of MMR in CAG expansion may shed light on potential avenues for therapeutic intervention in HD.<\/jats:p>","DOI":"10.3233\/jhd-200438","type":"journal-article","created":{"date-parts":[[2021,2,9]],"date-time":"2021-02-09T21:44:42Z","timestamp":1612907082000},"page":"75-94","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":125,"title":["DNA Mismatch Repair and its Role in Huntington\u2019s Disease"],"prefix":"10.1177","volume":"10","author":[{"given":"Ravi R.","family":"Iyer","sequence":"first","affiliation":[{"name":"CHDI Management\/CHDI Foundation, Princeton, NJ, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Pluciennik","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Thomas Jefferson University, Philadelphia, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2021,2,9]]},"reference":[{"key":"ref001","unstructured":"FriedbergEC, WalkerGC, SiedeW, WoodRD, SchultzRA, EllenbergerT. DNA Repair and Mutagenesis. 2nd ed. Washington, D.C.: ASM Press; 2006."},{"key":"ref002","doi-asserted-by":"publisher","DOI":"10.1101\/sqb.2009.74.027"},{"key":"ref003","doi-asserted-by":"publisher","DOI":"10.1021\/cr0404794"},{"key":"ref004","doi-asserted-by":"publisher","DOI":"10.1016\/j.mad.2008.02.012"},{"key":"ref005","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a012633"},{"key":"ref006","doi-asserted-by":"publisher","DOI":"10.1002\/anie.201601412"},{"key":"ref007","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-genet-112414-054722"},{"key":"ref008","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2019.03.009"},{"key":"ref009","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2015.11.019"},{"key":"ref010","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a022657"},{"key":"ref011","doi-asserted-by":"publisher","DOI":"10.1126\/science.1056421"},{"key":"ref012","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2958.2006.05150.x"},{"key":"ref013","doi-asserted-by":"publisher","DOI":"10.1007\/s10689-016-9882-8"},{"key":"ref014","doi-asserted-by":"publisher","DOI":"10.1002\/path.5422"},{"key":"ref015","doi-asserted-by":"crossref","unstructured":"WimmerK, EtzlerJ. Constitutional mismatch repair-deficiency syndrome: have we so far seen only the tip of an iceberg? Hum Genet (2008;124(2):105\u201322.","DOI":"10.1007\/s00439-008-0542-4"},{"key":"ref016","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2015.11.018"},{"key":"ref017","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.26249"},{"key":"ref018","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2003.04.060"},{"key":"ref019","doi-asserted-by":"publisher","DOI":"10.1172\/JCI122010"},{"key":"ref020","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2015.11.011"},{"key":"ref021","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.immunol.26.021607.090236"},{"key":"ref022","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.biochem.76.061705.090740"},{"key":"ref023","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2012.07.006"},{"key":"ref024","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1115361109"},{"key":"ref025","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2015.11.020"},{"key":"ref026","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-biochem-060614-034010"},{"key":"ref027","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(16)30350-7"},{"key":"ref028","doi-asserted-by":"publisher","DOI":"10.1242\/dmm.031930"},{"key":"ref029","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.R115.660142"},{"key":"ref030","first-page":"77","volume":"31","author":"Streisinger G","year":"1966","journal-title":"This paper is dedicated to Professor Theodosius Dobzhansky on the occasion of his 66th birthday. Cold Spring Harb Symp Quant Biol"},{"key":"ref031","doi-asserted-by":"publisher","DOI":"10.1016\/0958-1669(92)90005-4"},{"issue":"3","key":"ref032","first-page":"203","volume":"4","author":"Levinson G","year":"1987","journal-title":"Mol Biol Evol"},{"key":"ref033","doi-asserted-by":"publisher","DOI":"10.2741\/2427"},{"key":"ref034","doi-asserted-by":"publisher","DOI":"10.1007\/s12551-016-0210-1"},{"key":"ref035","doi-asserted-by":"publisher","DOI":"10.1002\/j.1460-2075.1990.tb07406.x"},{"key":"ref036","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(88)90014-1"},{"key":"ref037","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.27.16783"},{"key":"ref038","doi-asserted-by":"publisher","DOI":"10.1126\/science.8036515"},{"key":"ref039","doi-asserted-by":"publisher","DOI":"10.1021\/bi9601013"},{"key":"ref040","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1003866"},{"key":"ref041","doi-asserted-by":"publisher","DOI":"10.1038\/nsmb.2175"},{"key":"ref042","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2007.04.018"},{"key":"ref043","doi-asserted-by":"publisher","DOI":"10.1038\/35037523"},{"key":"ref044","doi-asserted-by":"publisher","DOI":"10.1038\/35037509"},{"key":"ref045","doi-asserted-by":"publisher","DOI":"10.1016\/j.mam.2019.03.001"},{"key":"ref046","doi-asserted-by":"publisher","DOI":"10.1038\/ng0995-64"},{"key":"ref047","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90319-4"},{"key":"ref048","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80433-X"},{"key":"ref049","doi-asserted-by":"publisher","DOI":"10.1038\/15544"},{"key":"ref050","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.31.21659"},{"key":"ref051","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M900908200"},{"key":"ref052","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.C109.014977"},{"key":"ref053","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80490-0"},{"key":"ref054","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.48.32049"},{"key":"ref055","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M202282200"},{"key":"ref056","doi-asserted-by":"publisher","DOI":"10.1128\/jb.176.17.5393-5400.1994"},{"key":"ref057","doi-asserted-by":"publisher","DOI":"10.1016\/S0968-0004(99)01503-0"},{"key":"ref058","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/20.19.5521"},{"key":"ref059","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2010.01.010"},{"key":"ref060","doi-asserted-by":"publisher","DOI":"10.1107\/S2053230X15010183"},{"key":"ref061","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.275.13.9863"},{"key":"ref062","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0800276105"},{"key":"ref063","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2010.08.006"},{"key":"ref064","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.16.9837"},{"key":"ref065","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M105076200"},{"key":"ref066","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-9822(06)00337-X"},{"key":"ref067","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M110.104125"},{"key":"ref068","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M407545200"},{"key":"ref069","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0912250106"},{"key":"ref070","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M114.552190"},{"key":"ref071","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M308738200"},{"key":"ref072","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.RA118.005439"},{"key":"ref073","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1807319115"},{"key":"ref074","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2006.05.039"},{"key":"ref075","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1010662107"},{"key":"ref076","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1702561114"},{"key":"ref077","doi-asserted-by":"publisher","DOI":"10.1038\/ng0398-276"},{"key":"ref078","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1008902"},{"key":"ref079","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddh186"},{"key":"ref080","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1003930"},{"key":"ref081","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M111854200"},{"key":"ref082","doi-asserted-by":"publisher","DOI":"10.1016\/S1097-2765(03)00428-3"},{"key":"ref083","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2005.06.027"},{"key":"ref084","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M109.021287"},{"key":"ref085","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2004.06.016"},{"key":"ref086","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M509701200"},{"key":"ref087","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0903654106"},{"key":"ref088","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.16.10917"},{"key":"ref089","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M113.534644"},{"key":"ref090","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M113.533810"},{"key":"ref091","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1914718117"},{"key":"ref092","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.24.13914"},{"key":"ref093","doi-asserted-by":"publisher","DOI":"10.1038\/ng931"},{"key":"ref094","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.15.1697"},{"key":"ref095","doi-asserted-by":"publisher","DOI":"10.1038\/s41586-020-2592-2"},{"key":"ref096","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.00855-07"},{"key":"ref097","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.21.12404"},{"key":"ref098","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-9822(00)00314-6"},{"key":"ref099","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-05-0742"},{"key":"ref100","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-05-2528"},{"key":"ref101","doi-asserted-by":"publisher","DOI":"10.1038\/s41436-018-0405-x"},{"key":"ref102","doi-asserted-by":"publisher","DOI":"10.1038\/71643"},{"key":"ref103","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2019.06.036"},{"key":"ref104","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1007719"},{"key":"ref105","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.45.32368"},{"key":"ref106","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M908768199"},{"key":"ref107","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/30.3.711"},{"key":"ref108","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M609989200"},{"key":"ref109","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1004327"},{"key":"ref110","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.15155"},{"key":"ref111","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkz441"},{"key":"ref112","doi-asserted-by":"publisher","DOI":"10.1016\/j.tibs.2007.04.003"},{"key":"ref113","doi-asserted-by":"publisher","DOI":"10.1002\/mc.20507"},{"key":"ref114","doi-asserted-by":"publisher","DOI":"10.1039\/c1cs15153c"},{"key":"ref115","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-genet-072610-155046"},{"key":"ref116","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1311325110"},{"key":"ref117","doi-asserted-by":"publisher","DOI":"10.1038\/nature02585"},{"key":"ref118","doi-asserted-by":"publisher","DOI":"10.1016\/j.tibs.2012.03.001"},{"key":"ref119","doi-asserted-by":"publisher","DOI":"10.1186\/s13578-020-0379-7"},{"key":"ref120","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2013.03.025"},{"key":"ref121","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1806355115"},{"key":"ref122","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.RA118.002839"},{"key":"ref123","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1106696109"},{"key":"ref124","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1015914108"},{"key":"ref125","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M115.713271"},{"key":"ref126","doi-asserted-by":"publisher","DOI":"10.1021\/bi7024639"},{"key":"ref127","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2018.09.001"},{"key":"ref128","doi-asserted-by":"publisher","DOI":"10.1080\/15384047.2017.1385680"},{"key":"ref129","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2015.11.008"},{"key":"ref130","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.18.00474"},{"key":"ref131","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0800048105"},{"key":"ref132","doi-asserted-by":"publisher","DOI":"10.1016\/S0169-328X(97)00311-2"},{"key":"ref133","doi-asserted-by":"publisher","DOI":"10.1016\/S0891-5849(00)00172-6"},{"key":"ref134","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2012.10.006"},{"key":"ref135","doi-asserted-by":"publisher","DOI":"10.1126\/science.1260419"},{"key":"ref136","doi-asserted-by":"publisher","DOI":"10.1016\/S0306-4522(99)00380-2"},{"key":"ref137","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2013.04.015"},{"key":"ref138","doi-asserted-by":"publisher","DOI":"10.1016\/j.redox.2018.01.008"},{"key":"ref139","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2014.06.034"},{"key":"ref140","doi-asserted-by":"publisher","DOI":"10.1089\/ars.2010.3474"},{"key":"ref141","doi-asserted-by":"publisher","DOI":"10.1016\/j.mad.2012.01.005"},{"key":"ref142","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2020.105021"},{"key":"ref143","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M108618200"},{"issue":"24","key":"ref144","first-page":"7087","volume":"24","author":"Zhao XN","year":"2015","journal-title":"Hum Mol Genet"},{"key":"ref145","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M112.356758"},{"key":"ref146","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gky143"},{"key":"ref147","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2012.01.002"},{"key":"ref148","doi-asserted-by":"publisher","DOI":"10.1038\/s41582-020-0389-4"},{"key":"ref149","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2015.12.018"},{"key":"ref150","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddg352"},{"key":"ref151","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddm054"},{"key":"ref152","doi-asserted-by":"publisher","DOI":"10.1038\/ng0494-409"},{"key":"ref153","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddp242"},{"key":"ref154","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2019.04.007"},{"key":"ref155","doi-asserted-by":"publisher","DOI":"10.1016\/j.ebiom.2019.09.020"},{"key":"ref156","doi-asserted-by":"publisher","DOI":"10.1038\/s41436-020-0917-z"},{"key":"ref157","doi-asserted-by":"publisher","DOI":"10.1101\/2020.07.23.218347"},{"key":"ref158","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddy375"},{"key":"ref159","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2015.07.003"},{"key":"ref160","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24656"},{"key":"ref161","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awz115"},{"key":"ref162","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddx286"},{"key":"ref163","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(17)30161-8"},{"key":"ref164","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2016.01.001"},{"key":"ref165","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.92.24.11019"},{"key":"ref166","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.31.19532"},{"key":"ref167","doi-asserted-by":"publisher","DOI":"10.1038\/70598"},{"key":"ref168","doi-asserted-by":"publisher","DOI":"10.1038\/86906"},{"key":"ref169","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.2.191"},{"key":"ref170","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddg056"},{"key":"ref171","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/cdg202"},{"key":"ref172","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.24.2.629-637.2004"},{"key":"ref173","doi-asserted-by":"publisher","DOI":"10.1038\/nsmb965"},{"key":"ref174","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-006-0164-7"},{"key":"ref175","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2008.09.014"},{"key":"ref176","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1000482"},{"key":"ref177","doi-asserted-by":"publisher","DOI":"10.3233\/JHD-170282"},{"key":"ref178","doi-asserted-by":"publisher","DOI":"10.1098\/rsif.2013.0605"},{"key":"ref179","doi-asserted-by":"crossref","unstructured":"MoralesF, VasquezM, CorralesE, Vindas-SmithR, Santamaria-UlloaC, ZhangB, et al. Longitudinal increases in somatic mosaicism of the expanded CTG repeat in myotonic dystrophy type 1 are associated with variation in age-at-onset. Hum Mol Genet. 2020.","DOI":"10.1093\/hmg\/ddaa123"},{"key":"ref180","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2015.01.004"},{"key":"ref181","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms13272"},{"key":"ref182","doi-asserted-by":"publisher","DOI":"10.1038\/s41588-019-0575-8"},{"key":"ref183","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddaa139"},{"key":"ref184","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2018.08.010"},{"key":"ref185","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-020-15901-w"},{"key":"ref186","doi-asserted-by":"publisher","DOI":"10.1002\/mc.20488"},{"key":"ref187","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0909740107"},{"key":"ref188","doi-asserted-by":"publisher","DOI":"10.4161\/cc.10.4.14729"},{"key":"ref189","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.51.2.315"},{"key":"ref190","doi-asserted-by":"publisher","DOI":"10.1016\/0022-2836(67)90018-6"},{"key":"ref191","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1105461108"},{"key":"ref192","doi-asserted-by":"publisher","DOI":"10.1038\/nsmb959"},{"key":"ref193","doi-asserted-by":"publisher","DOI":"10.1038\/nsmb.1638"},{"key":"ref194","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2011.10.020"},{"key":"ref195","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.31.19895"},{"key":"ref196","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.11.7474"},{"key":"ref197","doi-asserted-by":"publisher","DOI":"10.1126\/science.7973637"},{"key":"ref198","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M309025200"},{"key":"ref199","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0909087107"},{"key":"ref200","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M112.420398"},{"key":"ref201","doi-asserted-by":"publisher","DOI":"10.1038\/cr.2016.66"},{"key":"ref202","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms12465"},{"key":"ref203","doi-asserted-by":"publisher","DOI":"10.1038\/nature05778"},{"key":"ref204","doi-asserted-by":"publisher","DOI":"10.1016\/bs.enz.2019.08.002"},{"key":"ref205","doi-asserted-by":"crossref","unstructured":"LoupeJM, PintoRM, KimKH, GillisT, MysoreJS, AndrewMA, et al. Promotion of somatic CAG repeat expansion by Fan1 knock-out in Huntington\u2019s disease knock-in mice is blocked by Mlh1 knock-out. Hum Mol Genet. 2020.","DOI":"10.1093\/hmg\/ddaa196"},{"key":"ref206","doi-asserted-by":"publisher","DOI":"10.1016\/j.dnarep.2018.07.001"},{"key":"ref207","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2010.06.023"},{"key":"ref208","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2010.06.021"},{"key":"ref209","doi-asserted-by":"publisher","DOI":"10.1111\/gtc.12748"},{"key":"ref210","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2020.05.012"},{"key":"ref211","doi-asserted-by":"publisher","DOI":"10.1038\/s41586-020-2308-7"},{"key":"ref212","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddh325"},{"key":"ref213","doi-asserted-by":"publisher","DOI":"10.1002\/humu.22464"},{"key":"ref214","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddt386"},{"key":"ref215","doi-asserted-by":"publisher","DOI":"10.1038\/srep11020"},{"key":"ref216","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gks810"},{"key":"ref217","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M112.391961"},{"key":"ref218","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1003280"},{"key":"ref219","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddr099"},{"key":"ref220","doi-asserted-by":"publisher","DOI":"10.1007\/978-94-007-4572-8_17"},{"key":"ref221","doi-asserted-by":"publisher","DOI":"10.1172\/JCI99629"},{"key":"ref222","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2347"}],"container-title":["Journal of Huntington's Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.3233\/JHD-200438","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.3233\/JHD-200438","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.3233\/JHD-200438","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T08:55:57Z","timestamp":1777452957000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.3233\/JHD-200438"}},"subtitle":[],"editor":[{"given":"Lesley","family":"Jones","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]},{"given":"Christopher E.","family":"Pearson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]},{"given":"Vanessa","family":"Wheeler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2021,2,9]]},"references-count":222,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,2,9]]}},"alternative-id":["10.3233\/JHD-200438"],"URL":"https:\/\/doi.org\/10.3233\/jhd-200438","relation":{},"ISSN":["1879-6397","1879-6400"],"issn-type":[{"value":"1879-6397","type":"print"},{"value":"1879-6400","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,2,9]]}}}