{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T19:34:54Z","timestamp":1786995294064,"version":"build-2736575974"},"reference-count":164,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2025,12,1]],"date-time":"2025-12-01T00:00:00Z","timestamp":1764547200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,12,1]],"date-time":"2025-12-01T00:00:00Z","timestamp":1764547200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,9,5]],"date-time":"2025-09-05T00:00:00Z","timestamp":1757030400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000887","name":"Huntington&apos;s Disease Society of America","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000887","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000245","name":"Michael Smith Health Research BC","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000245","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008630","name":"Children&apos;s Health Research Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008630","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["cell.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Therapy Nucleic Acids"],"published-print":{"date-parts":[[2025,12]]},"DOI":"10.1016\/j.omtn.2025.102710","type":"journal-article","created":{"date-parts":[[2025,9,6]],"date-time":"2025-09-06T14:58:21Z","timestamp":1757170701000},"page":"102710","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":2,"title":["Molecular and imaging biomarker responses to brain mutant HTT lowering in a mouse model of Huntington disease"],"prefix":"10.1016","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3877-455X","authenticated-orcid":false,"given":"Nicholas S.","family":"Caron","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianjia","family":"Fan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Da","family":"Ma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Yung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seunghyun","family":"Ko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kirsten","family":"Bale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Anderson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piotr","family":"Kozlowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mahmoud A.","family":"Pouladi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cheryl L.","family":"Wellington","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael R.","family":"Hayden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.omtn.2025.102710_bib1","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1016\/S1474-4422(17)30124-2","article-title":"Neurofilament light protein in blood as a potential biomarker of neurodegeneration in Huntington's disease: a retrospective cohort analysis","volume":"16","author":"Byrne","year":"2017","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib2","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.aat7108","article-title":"Evaluation of mutant huntingtin and neurofilament proteins as potential markers in Huntington's disease","volume":"10","author":"Byrne","year":"2018","journal-title":"Sci. Transl. Med."},{"key":"10.1016\/j.omtn.2025.102710_bib3","doi-asserted-by":"crossref","first-page":"e717","DOI":"10.1212\/WNL.0000000000005005","article-title":"Neurofilament light protein in blood predicts regional atrophy in Huntington disease","volume":"90","author":"Johnson","year":"2018","journal-title":"Neurology"},{"key":"10.1016\/j.omtn.2025.102710_bib4","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.abc2888","article-title":"Mutant huntingtin and neurofilament light have distinct longitudinal dynamics in Huntington's disease","volume":"12","author":"Rodrigues","year":"2020","journal-title":"Sci. Transl. Med."},{"key":"10.1016\/j.omtn.2025.102710_bib5","doi-asserted-by":"crossref","first-page":"2108","DOI":"10.1007\/s00415-023-12109-y","article-title":"Plasma GFAP and its association with disease severity in Huntington's disease","volume":"271","author":"Tan","year":"2024","journal-title":"J. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib6","doi-asserted-by":"crossref","DOI":"10.3389\/fneur.2021.779890","article-title":"Evaluation of Blood Glial Fibrillary Acidic Protein as a Potential Marker in Huntington's Disease","volume":"12","author":"You","year":"2021","journal-title":"Front. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib7","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1016\/S1474-4422(09)70170-X","article-title":"Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data","volume":"8","author":"Tabrizi","year":"2009","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib8","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1016\/S1474-4422(11)70263-0","article-title":"Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data","volume":"11","author":"Tabrizi","year":"2012","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib9","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/S1474-4422(10)70276-3","article-title":"Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis","volume":"10","author":"Tabrizi","year":"2011","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib10","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1016\/S1474-4422(13)70088-7","article-title":"Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data","volume":"12","author":"Tabrizi","year":"2013","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib11","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes","volume":"72","author":"MacDonald","year":"1993","journal-title":"Cell"},{"issue":"Pt 6","key":"10.1016\/j.omtn.2025.102710_bib12","doi-asserted-by":"crossref","first-page":"2085","DOI":"10.1093\/brain\/119.6.2085","article-title":"Striatal glucose metabolism and dopamine D2 receptor binding in asymptomatic gene carriers and patients with Huntington's disease","volume":"119","author":"Antonini","year":"1996","journal-title":"Brain"},{"key":"10.1016\/j.omtn.2025.102710_bib13","doi-asserted-by":"crossref","first-page":"1161","DOI":"10.1001\/archneur.1992.00530350075022","article-title":"Serial changes of cerebral glucose metabolism and caudate size in persons at risk for Huntington's disease","volume":"49","author":"Grafton","year":"1992","journal-title":"Arch. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib14","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/j.cmet.2006.10.004","article-title":"Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration","volume":"4","author":"Weydt","year":"2006","journal-title":"Cell Metab."},{"key":"10.1016\/j.omtn.2025.102710_bib15","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.cell.2006.09.015","article-title":"Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration","volume":"127","author":"Cui","year":"2006","journal-title":"Cell"},{"key":"10.1016\/j.omtn.2025.102710_bib16","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1126\/science.1059581","article-title":"Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease","volume":"293","author":"Zuccato","year":"2001","journal-title":"Science"},{"key":"10.1016\/j.omtn.2025.102710_bib17","doi-asserted-by":"crossref","first-page":"2238","DOI":"10.1126\/science.1072613","article-title":"Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease","volume":"296","author":"Dunah","year":"2002","journal-title":"Science"},{"key":"10.1016\/j.omtn.2025.102710_bib18","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.cell.2004.06.018","article-title":"Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules","volume":"118","author":"Gauthier","year":"2004","journal-title":"Cell"},{"key":"10.1016\/j.omtn.2025.102710_bib19","doi-asserted-by":"crossref","first-page":"6298","DOI":"10.1523\/JNEUROSCI.2033-12.2013","article-title":"Mutant Huntingtin alters retrograde transport of TrkB receptors in striatal dendrites","volume":"33","author":"Liot","year":"2013","journal-title":"J. Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib20","doi-asserted-by":"crossref","first-page":"3837","DOI":"10.1093\/hmg\/ddn281","article-title":"Phosphorylation of mutant huntingtin at S421 restores anterograde and retrograde transport in neurons","volume":"17","author":"Zala","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib21","doi-asserted-by":"crossref","first-page":"1937","DOI":"10.1093\/hmg\/ddr075","article-title":"Mutant huntingtin causes defective actin remodeling during stress: defining a new role for transglutaminase 2 in neurodegenerative disease","volume":"20","author":"Munsie","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib22","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1093\/hmg\/ddu460","article-title":"A huntingtin-mediated fast stress response halting endosomal trafficking is defective in Huntington's disease","volume":"24","author":"Nath","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib23","doi-asserted-by":"crossref","first-page":"2600","DOI":"10.1093\/hmg\/ddm217","article-title":"Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity","volume":"16","author":"Atwal","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib24","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.jchemneu.2004.02.005","article-title":"Differential loss of striatal projection systems in Huntington's disease: a quantitative immunohistochemical study","volume":"27","author":"Deng","year":"2004","journal-title":"J. Chem. Neuroanat."},{"key":"10.1016\/j.omtn.2025.102710_bib25","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1016\/S0306-4522(00)00008-7","article-title":"The pattern of neurodegeneration in Huntington's disease: a comparative study of cannabinoid, dopamine, adenosine and GABA(A) receptor alterations in the human basal ganglia in Huntington's disease","volume":"97","author":"Glass","year":"2000","journal-title":"Neuroscience"},{"key":"10.1016\/j.omtn.2025.102710_bib26","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/0304-3940(91)90583-F","article-title":"Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease","volume":"133","author":"Hedreen","year":"1991","journal-title":"Neurosci. Lett."},{"key":"10.1016\/j.omtn.2025.102710_bib27","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1002\/ana.24694","article-title":"Globus pallidus degeneration and clinicopathological features of Huntington disease","volume":"80","author":"Singh-Bains","year":"2016","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib28","doi-asserted-by":"crossref","first-page":"17204","DOI":"10.1073\/pnas.0708285104","article-title":"Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits","volume":"104","author":"DiFiglia","year":"2007","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.omtn.2025.102710_bib29","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1002\/ana.21569","article-title":"Sustained effects of nonallele-specific Huntingtin silencing","volume":"65","author":"Drouet","year":"2009","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib30","doi-asserted-by":"crossref","first-page":"1053","DOI":"10.1038\/mt.2009.17","article-title":"Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice","volume":"17","author":"Boudreau","year":"2009","journal-title":"Mol. Ther."},{"key":"10.1016\/j.omtn.2025.102710_bib31","doi-asserted-by":"crossref","first-page":"1031","DOI":"10.1016\/j.neuron.2012.05.009","article-title":"Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis","volume":"74","author":"Kordasiewicz","year":"2012","journal-title":"Neuron"},{"key":"10.1016\/j.omtn.2025.102710_bib32","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1089\/hum.2013.200","article-title":"Silencing mutant huntingtin by adeno-associated virus-mediated RNA interference ameliorates disease manifestations in the YAC128 mouse model of Huntington's disease","volume":"25","author":"Stanek","year":"2014","journal-title":"Hum. Gene Ther."},{"key":"10.1016\/j.omtn.2025.102710_bib33","doi-asserted-by":"crossref","first-page":"217","DOI":"10.3233\/JHD-130057","article-title":"Antisense oligonucleotide-mediated correction of transcriptional dysregulation is correlated with behavioral benefits in the YAC128 mouse model of Huntington's disease","volume":"2","author":"Stanek","year":"2013","journal-title":"J. Huntingtons Dis."},{"key":"10.1016\/j.omtn.2025.102710_bib34","doi-asserted-by":"crossref","first-page":"5820","DOI":"10.1073\/pnas.0501507102","article-title":"RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model","volume":"102","author":"Harper","year":"2005","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.omtn.2025.102710_bib35","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0171127","article-title":"The expanded CAG repeat in the huntingtin gene as target for therapeutic RNA modulation throughout the HD mouse brain","volume":"12","author":"Datson","year":"2017","journal-title":"PLoS One"},{"key":"10.1016\/j.omtn.2025.102710_bib36","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1016\/j.ymthe.2005.05.006","article-title":"Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6\/1 Huntington's disease transgenic mice","volume":"12","author":"Rodriguez-Lebron","year":"2005","journal-title":"Mol. Ther."},{"key":"10.1016\/j.omtn.2025.102710_bib37","first-page":"36","article-title":"Potent and sustained huntingtin lowering via AAV5 encoding miRNA preserves striatal volume and cognitive function in a humanized mouse model of Huntington disease","volume":"48","author":"Caron","year":"2020","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.omtn.2025.102710_bib38","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.aar3959","article-title":"Huntingtin suppression restores cognitive function in a mouse model of Huntington's disease","volume":"10","author":"Southwell","year":"2018","journal-title":"Sci. Transl. Med."},{"key":"10.1016\/j.omtn.2025.102710_bib39","doi-asserted-by":"crossref","first-page":"1131","DOI":"10.1038\/s41591-019-0478-3","article-title":"Allele-selective transcriptional repression of mutant HTT for the treatment of Huntington's disease","volume":"25","author":"Zeitler","year":"2019","journal-title":"Nat. Med."},{"key":"10.1016\/j.omtn.2025.102710_bib40","doi-asserted-by":"crossref","first-page":"2298","DOI":"10.1093\/brain\/awac458","article-title":"AAV5-miHTT-mediated huntingtin lowering improves brain health in a Huntington's disease mouse model","volume":"146","author":"Thomson","year":"2023","journal-title":"Brain"},{"key":"10.1016\/j.omtn.2025.102710_bib41","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0024308","article-title":"Targeting several CAG expansion diseases by a single antisense oligonucleotide","volume":"6","author":"Evers","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.omtn.2025.102710_bib42","doi-asserted-by":"crossref","first-page":"5578","DOI":"10.1093\/nar\/gkr156","article-title":"Inhibition of mutant huntingtin expression by RNA duplex targeting expanded CAG repeats","volume":"39","author":"Fiszer","year":"2011","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.omtn.2025.102710_bib43","doi-asserted-by":"crossref","first-page":"1112","DOI":"10.1016\/j.ajhg.2019.10.011","article-title":"A Comprehensive Haplotype-Targeting Strategy for Allele-Specific HTT Suppression in Huntington Disease","volume":"105","author":"Kay","year":"2019","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib44","doi-asserted-by":"crossref","first-page":"1759","DOI":"10.1038\/mt.2015.128","article-title":"Huntingtin Haplotypes Provide Prioritized Target Panels for Allele-specific Silencing in Huntington Disease Patients of European Ancestry","volume":"23","author":"Kay","year":"2015","journal-title":"Mol. Ther."},{"key":"10.1016\/j.omtn.2025.102710_bib45","doi-asserted-by":"crossref","first-page":"2178","DOI":"10.1038\/mt.2011.201","article-title":"Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene\/allele-specific silencing of mutant huntingtin","volume":"19","author":"Carroll","year":"2011","journal-title":"Mol. Ther."},{"key":"10.1016\/j.omtn.2025.102710_bib46","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1126\/science.7618107","article-title":"Inactivation of the mouse Huntington's disease gene homolog Hdh","volume":"269","author":"Duyao","year":"1995","journal-title":"Science"},{"key":"10.1016\/j.omtn.2025.102710_bib47","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1038\/ng1095-155","article-title":"Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue","volume":"11","author":"Zeitlin","year":"1995","journal-title":"Nat. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib48","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1016\/0092-8674(95)90542-1","article-title":"Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes","volume":"81","author":"Nasir","year":"1995","journal-title":"Cell"},{"key":"10.1016\/j.omtn.2025.102710_bib49","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1006846","article-title":"Elimination of huntingtin in the adult mouse leads to progressive behavioral deficits, bilateral thalamic calcification, and altered brain iron homeostasis","volume":"13","author":"Dietrich","year":"2017","journal-title":"PLoS Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib50","doi-asserted-by":"crossref","DOI":"10.26508\/lsa.202402571","article-title":"Global huntingtin knockout in adult mice leads to fatal neurodegeneration that spares the pancreas","volume":"7","author":"Bragg","year":"2024","journal-title":"Life Sci. Alliance"},{"key":"10.1016\/j.omtn.2025.102710_bib53","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1002\/mds.870110204","article-title":"Unified Huntington's disease rating scale: reliability and consistency","volume":"11","author":"Huntington","year":"1996","journal-title":"Movement disorders"},{"key":"10.1016\/j.omtn.2025.102710_bib54","doi-asserted-by":"crossref","first-page":"2495","DOI":"10.1212\/WNL.0000000000004743","article-title":"Motor, cognitive, and functional declines contribute to a single progressive factor in early HD","volume":"89","author":"Schobel","year":"2017","journal-title":"Neurology"},{"key":"10.1016\/j.omtn.2025.102710_bib55","doi-asserted-by":"crossref","DOI":"10.3389\/fneur.2021.712555","article-title":"Volumetric MRI-Based Biomarkers in Huntington's Disease: An Evidentiary Review","volume":"12","author":"Kinnunen","year":"2021","journal-title":"Front. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib56","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1136\/jnnp.2007.128728","article-title":"Detection of Huntington's disease decades before diagnosis: the Predict-HD study","volume":"79","author":"Paulsen","year":"2008","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"10.1016\/j.omtn.2025.102710_bib57","doi-asserted-by":"crossref","first-page":"1193","DOI":"10.1016\/S1474-4422(14)70238-8","article-title":"Prediction of manifest Huntington's disease with clinical and imaging measures: a prospective observational study","volume":"13","author":"Paulsen","year":"2014","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib58","doi-asserted-by":"crossref","DOI":"10.1212\/NXG.0000000000000617","article-title":"Revealing the Timeline of Structural MRI Changes in Premanifest to Manifest Huntington Disease","volume":"7","author":"Wijeratne","year":"2021","journal-title":"Neurol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib59","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1016\/S1474-4422(20)30143-5","article-title":"Biological and clinical characteristics of gene carriers far from predicted onset in the Huntington's disease Young Adult Study (HD-YAS): a cross-sectional analysis","volume":"19","author":"Scahill","year":"2020","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib60","doi-asserted-by":"crossref","DOI":"10.1016\/j.nbd.2021.105311","article-title":"Plasma neurofilament light chain predicts cerebellar atrophy and clinical progression in spinocerebellar ataxia","volume":"153","author":"Coarelli","year":"2021","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.omtn.2025.102710_bib61","doi-asserted-by":"crossref","first-page":"2129","DOI":"10.1007\/s00415-019-09389-8","article-title":"NFL is a marker of treatment response in children with SMA treated with nusinersen","volume":"266","author":"Olsson","year":"2019","journal-title":"J. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib62","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1001\/jamaneurol.2016.5398","article-title":"Diagnostic and Prognostic Biomarkers in Amyotrophic Lateral Sclerosis: Neurofilament Light Chain Levels in Definite Subtypes of Disease","volume":"74","author":"Gaiani","year":"2017","journal-title":"JAMA Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib63","doi-asserted-by":"crossref","first-page":"2302","DOI":"10.1212\/WNL.0000000000004029","article-title":"Neurofilament markers for ALS correlate with extent of upper and lower motor neuron disease","volume":"88","author":"Poesen","year":"2017","journal-title":"Neurology"},{"key":"10.1016\/j.omtn.2025.102710_bib64","doi-asserted-by":"crossref","first-page":"e1104","DOI":"10.1212\/WNL.0000000000008088","article-title":"Blood NfL: A biomarker for disease severity and progression in Parkinson disease","volume":"93","author":"Lin","year":"2019","journal-title":"Neurology"},{"key":"10.1016\/j.omtn.2025.102710_bib65","doi-asserted-by":"crossref","first-page":"1999","DOI":"10.1002\/mds.28206","article-title":"Validation of Serum Neurofilament Light Chain as a Biomarker of Parkinson's Disease Progression","volume":"35","author":"Mollenhauer","year":"2020","journal-title":"Mov. Disord."},{"key":"10.1016\/j.omtn.2025.102710_bib66","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1001\/jamaneurol.2016.6117","article-title":"Association of Plasma Neurofilament Light With Neurodegeneration in Patients With Alzheimer Disease","volume":"74","author":"Mattsson","year":"2017","journal-title":"JAMA Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib67","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1038\/s41591-018-0304-3","article-title":"Serum neurofilament dynamics predicts neurodegeneration and clinical progression in presymptomatic Alzheimer's disease","volume":"25","author":"Preische","year":"2019","journal-title":"Nat. Med."},{"key":"10.1016\/j.omtn.2025.102710_bib68","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-017-14179-1","article-title":"Neurofilament light protein in CSF and blood is associated with neurodegeneration and disease severity in Huntington's disease R6\/2 mice","volume":"7","author":"Soylu-Kucharz","year":"2017","journal-title":"Sci. Rep."},{"key":"10.1016\/j.omtn.2025.102710_bib69","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1523\/JNEUROSCI.1865-20.2020","article-title":"Mutant huntingtin is cleared from the brain via active mechanisms in Huntington disease","volume":"41","author":"Caron","year":"2021","journal-title":"J. Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib70","article-title":"Translatable plasma and CSF biomarkers for use in mouse models of Huntington's disease","volume":"6","author":"Bondulich","year":"2024","journal-title":"Brain Commun."},{"key":"10.1016\/j.omtn.2025.102710_bib73","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1177\/1352458513490544","article-title":"Immunosuppressive therapy reduces axonal damage in progressive multiple sclerosis","volume":"20","author":"Axelsson","year":"2014","journal-title":"Mult. Scler."},{"key":"10.1016\/j.omtn.2025.102710_bib74","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1002\/ana.22247","article-title":"Axonal damage in relapsing multiple sclerosis is markedly reduced by natalizumab","volume":"69","author":"Gunnarsson","year":"2011","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib75","doi-asserted-by":"crossref","first-page":"2230","DOI":"10.1212\/WNL.0000000000004683","article-title":"Monitoring disease activity in multiple sclerosis using serum neurofilament light protein","volume":"89","author":"Novakova","year":"2017","journal-title":"Neurology"},{"key":"10.1016\/j.omtn.2025.102710_bib76","doi-asserted-by":"crossref","first-page":"1639","DOI":"10.1212\/WNL.0000000000001491","article-title":"Fingolimod and CSF neurofilament light chain levels in relapsing-remitting multiple sclerosis","volume":"84","author":"Kuhle","year":"2015","journal-title":"Neurology"},{"key":"10.1016\/j.omtn.2025.102710_bib77","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1177\/1352458516639384","article-title":"Cerebrospinal fluid biomarkers of inflammation and degeneration as measures of fingolimod efficacy in multiple sclerosis","volume":"23","author":"Novakova","year":"2017","journal-title":"Mult. Scler."},{"key":"10.1016\/j.omtn.2025.102710_bib78","doi-asserted-by":"crossref","first-page":"1499","DOI":"10.1212\/WNL.0000000000000361","article-title":"Natalizumab in progressive MS: results of an open-label, phase 2A, proof-of-concept trial","volume":"82","author":"Romme Christensen","year":"2014","journal-title":"Neurology"},{"key":"10.1016\/j.omtn.2025.102710_bib79","doi-asserted-by":"crossref","first-page":"1068","DOI":"10.1136\/jnnp-2018-320033","article-title":"Neurofilaments and tau in CSF in an infant with SMA type 1 treated with nusinersen","volume":"90","author":"Winter","year":"2019","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"10.1016\/j.omtn.2025.102710_bib80","doi-asserted-by":"crossref","first-page":"1099","DOI":"10.1056\/NEJMoa2204705","article-title":"Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS","volume":"387","author":"Miller","year":"2022","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.omtn.2025.102710_bib81","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1186\/s40035-024-00443-8","article-title":"Elevated plasma and CSF neurofilament light chain concentrations are stabilized in response to mutant huntingtin lowering in the brains of Huntington's disease mice","volume":"13","author":"Caron","year":"2024","journal-title":"Transl. Neurodegener."},{"key":"10.1016\/j.omtn.2025.102710_bib82","doi-asserted-by":"crossref","first-page":"1471","DOI":"10.1001\/jamaneurol.2021.3671","article-title":"Differences Between Plasma and Cerebrospinal Fluid Glial Fibrillary Acidic Protein Levels Across the Alzheimer Disease Continuum","volume":"78","author":"Benedet","year":"2021","journal-title":"JAMA Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib83","doi-asserted-by":"crossref","DOI":"10.3390\/biom12020222","article-title":"Neuroinflammation Is Associated with GFAP and sTREM2 Levels in Multiple Sclerosis","volume":"12","author":"Azzolini","year":"2022","journal-title":"Biomolecules"},{"key":"10.1016\/j.omtn.2025.102710_bib84","doi-asserted-by":"crossref","DOI":"10.1001\/jamanetworkopen.2024.31959","article-title":"Plasma Biomarkers of Traumatic Brain Injury in Adolescents With Sport-Related Concussion","volume":"7","author":"Tabor","year":"2024","journal-title":"JAMA Netw. Open"},{"key":"10.1016\/j.omtn.2025.102710_bib85","doi-asserted-by":"crossref","first-page":"3053","DOI":"10.1093\/hmg\/ddq212","article-title":"In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects","volume":"19","author":"Faideau","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib86","doi-asserted-by":"crossref","DOI":"10.1016\/j.nbd.2024.106554","article-title":"Microglial proliferation and astrocytic protein alterations in the human Huntington's disease cortex","volume":"198","author":"Tan","year":"2024","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.omtn.2025.102710_bib87","doi-asserted-by":"crossref","DOI":"10.1016\/j.jns.2024.122979","article-title":"Cerebrospinal fluid glial fibrillary acidic protein, in contrast to amyloid beta protein, is associated with disease symptoms in Huntington's disease","volume":"459","author":"Korpela","year":"2024","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.omtn.2025.102710_bib88","doi-asserted-by":"crossref","first-page":"1555","DOI":"10.1093\/hmg\/ddg169","article-title":"Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease","volume":"12","author":"Slow","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib89","doi-asserted-by":"crossref","first-page":"4169","DOI":"10.1523\/JNEUROSCI.0590-05.2005","article-title":"Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease","volume":"25","author":"Van Raamsdonk","year":"2005","journal-title":"J. Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib90","doi-asserted-by":"crossref","first-page":"2219","DOI":"10.1093\/hmg\/dds037","article-title":"Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice","volume":"21","author":"Pouladi","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib91","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.brainresbull.2010.05.005","article-title":"Longitudinal analysis of the behavioural phenotype in YAC128 (C57BL\/6J) Huntington's disease transgenic mice","volume":"88","author":"Brooks","year":"2012","journal-title":"Brain Res. Bull."},{"key":"10.1016\/j.omtn.2025.102710_bib92","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1186\/1471-2202-7-80","article-title":"Wild-type huntingtin ameliorates striatal neuronal atrophy but does not prevent other abnormalities in the YAC128 mouse model of Huntington disease","volume":"7","author":"Van Raamsdonk","year":"2006","journal-title":"BMC Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib93","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/j.nbd.2011.03.018","article-title":"Natural history of disease in the YAC128 mouse reveals a discrete signature of pathology in Huntington disease","volume":"43","author":"Carroll","year":"2011","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.omtn.2025.102710_bib94","doi-asserted-by":"crossref","first-page":"2125","DOI":"10.1093\/hmg\/ddy119","article-title":"A whole brain longitudinal study in the YAC128 mouse model of Huntington's disease shows distinct trajectories of neurochemical, structural connectivity and volumetric changes","volume":"27","author":"Petrella","year":"2018","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib95","doi-asserted-by":"crossref","DOI":"10.1016\/j.nbd.2022.105652","article-title":"Cerebrospinal fluid mutant huntingtin is a biomarker for huntingtin lowering in the striatum of Huntington disease mice","volume":"166","author":"Caron","year":"2022","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.omtn.2025.102710_bib96","doi-asserted-by":"crossref","DOI":"10.1093\/braincomms\/fcad214","article-title":"Longitudinal imaging highlights preferential basal ganglia circuit atrophy in Huntington's disease","volume":"5","author":"Liu","year":"2023","journal-title":"Brain Commun."},{"key":"10.1016\/j.omtn.2025.102710_bib97","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1038\/nn.4256","article-title":"Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice","volume":"19","author":"Langfelder","year":"2016","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib98","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1016\/j.neuron.2020.06.021","article-title":"Cell Type-Specific Transcriptomics Reveals that Mutant Huntingtin Leads to Mitochondrial RNA Release and Neuronal Innate Immune Activation","volume":"107","author":"Lee","year":"2020","journal-title":"Neuron"},{"key":"10.1016\/j.omtn.2025.102710_bib99","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1038\/nature05453","article-title":"Genome-wide atlas of gene expression in the adult mouse brain","volume":"445","author":"Lein","year":"2007","journal-title":"Nature"},{"key":"10.1016\/j.omtn.2025.102710_bib100","doi-asserted-by":"crossref","first-page":"8868","DOI":"10.1038\/s41598-018-27293-5","article-title":"Brain Cell Type Specific Gene Expression and Co-expression Network Architectures","volume":"8","author":"McKenzie","year":"2018","journal-title":"Sci. Rep."},{"key":"10.1016\/j.omtn.2025.102710_bib101","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1016\/j.cell.2008.10.029","article-title":"Application of a translational profiling approach for the comparative analysis of CNS cell types","volume":"135","author":"Doyle","year":"2008","journal-title":"Cell"},{"key":"10.1016\/j.omtn.2025.102710_bib102","doi-asserted-by":"crossref","first-page":"9622","DOI":"10.1073\/pnas.1818042116","article-title":"Intrinsic mutant HTT-mediated defects in oligodendroglia cause myelination deficits and behavioral abnormalities in Huntington disease","volume":"116","author":"Ferrari Bardile","year":"2019","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.omtn.2025.102710_bib103","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1038\/nn.4216","article-title":"Adult mouse cortical cell taxonomy revealed by single cell transcriptomics","volume":"19","author":"Tasic","year":"2016","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib104","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1097\/00005072-198511000-00003","article-title":"Neuropathological classification of Huntington's disease","volume":"44","author":"Vonsattel","year":"1985","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib105","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1002\/ana.24406","article-title":"Cerebrovascular and blood-brain barrier impairments in Huntington's disease: Potential implications for its pathophysiology","volume":"78","author":"Drouin-Ouellet","year":"2015","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib106","doi-asserted-by":"crossref","first-page":"1365","DOI":"10.1016\/j.celrep.2017.04.021","article-title":"Huntington's Disease iPSC-Derived Brain Microvascular Endothelial Cells Reveal WNT-Mediated Angiogenic and Blood-Brain Barrier Deficits","volume":"19","author":"Lim","year":"2017","journal-title":"Cell Rep."},{"key":"10.1016\/j.omtn.2025.102710_bib107","doi-asserted-by":"crossref","first-page":"258","DOI":"10.3389\/fnmol.2019.00258","article-title":"The Role of Microglia and Astrocytes in Huntington's Disease","volume":"12","author":"Palpagama","year":"2019","journal-title":"Front. Mol. Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib108","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1038\/nn.3691","article-title":"Astrocyte Kir4.1 ion channel deficits contribute to neuronal dysfunction in Huntington's disease model mice","volume":"17","author":"Tong","year":"2014","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib109","doi-asserted-by":"crossref","first-page":"3453","DOI":"10.1523\/JNEUROSCI.3693-15.2016","article-title":"Dysfunctional Calcium and Glutamate Signaling in Striatal Astrocytes from Huntington's Disease Model Mice","volume":"36","author":"Jiang","year":"2016","journal-title":"J. Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib110","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1038\/nature21029","article-title":"Neurotoxic reactive astrocytes are induced by activated microglia","volume":"541","author":"Liddelow","year":"2017","journal-title":"Nature"},{"key":"10.1016\/j.omtn.2025.102710_bib111","doi-asserted-by":"crossref","first-page":"279","DOI":"10.3233\/JHD-240045","article-title":"Magnetic Resonance Imaging to Detect Structural Brain Changes in Huntington's Disease: A Review of Data from Mouse Models","volume":"13","author":"Hanrahan","year":"2024","journal-title":"J. Huntingtons Dis."},{"key":"10.1016\/j.omtn.2025.102710_bib112","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1016\/j.neuroimage.2015.11.073","article-title":"MUSE: MUlti-atlas region Segmentation utilizing Ensembles of registration algorithms and parameters, and locally optimal atlas selection","volume":"127","author":"Doshi","year":"2016","journal-title":"Neuroimage"},{"key":"10.1016\/j.omtn.2025.102710_bib113","doi-asserted-by":"crossref","DOI":"10.1016\/j.neuroimage.2020.117271","article-title":"Substantially thinner internal granular layer and reduced molecular layer surface in the cerebellar cortex of the Tc1 mouse model of down syndrome - a comprehensive morphometric analysis with active staining contrast-enhanced MRI","volume":"223","author":"Ma","year":"2020","journal-title":"Neuroimage"},{"key":"10.1016\/j.omtn.2025.102710_bib114","doi-asserted-by":"crossref","first-page":"11","DOI":"10.3389\/fnins.2019.00011","article-title":"Study the Longitudinal in vivo and Cross-Sectional ex vivo Brain Volume Difference for Disease Progression and Treatment Effect on Mouse Model of Tauopathy Using Automated MRI Structural Parcellation","volume":"13","author":"Ma","year":"2019","journal-title":"Front. Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib115","doi-asserted-by":"crossref","first-page":"20","DOI":"10.3389\/fninf.2017.00020","article-title":"Comparison of In Vivo and Ex Vivo MRI for the Detection of Structural Abnormalities in a Mouse Model of Tauopathy","volume":"11","author":"Holmes","year":"2017","journal-title":"Front. Neuroinform."},{"key":"10.1016\/j.omtn.2025.102710_bib116","doi-asserted-by":"crossref","first-page":"3654","DOI":"10.1093\/hmg\/ddw212","article-title":"An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes","volume":"25","author":"Southwell","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib117","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0162974","article-title":"Fully-Automated muMRI Morphometric Phenotyping of the Tc1 Mouse Model of Down Syndrome","volume":"11","author":"Powell","year":"2016","journal-title":"PLoS One"},{"key":"10.1016\/j.omtn.2025.102710_bib118","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1016\/j.neuroimage.2007.08.033","article-title":"Automated deformation analysis in the YAC128 Huntington disease mouse model","volume":"39","author":"Lerch","year":"2008","journal-title":"Neuroimage"},{"key":"10.1016\/j.omtn.2025.102710_bib119","doi-asserted-by":"crossref","first-page":"6","DOI":"10.3389\/fninf.2012.00006","article-title":"Wanted dead or alive? The tradeoff between in-vivo versus ex-vivo MR brain imaging in the mouse","volume":"6","author":"Lerch","year":"2012","journal-title":"Front. Neuroinform."},{"key":"10.1016\/j.omtn.2025.102710_bib120","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1002\/cne.21906","article-title":"Postmortem changes in the neuroanatomical characteristics of the primate brain: hippocampal formation","volume":"512","author":"Lavenex","year":"2009","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib121","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.biopsych.2005.06.003","article-title":"Brain structure in preclinical Huntington's disease","volume":"59","author":"Paulsen","year":"2006","journal-title":"Biol. Psychiatry"},{"key":"10.1016\/j.omtn.2025.102710_bib122","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1002\/mds.20979","article-title":"Diffusion tensor imaging in presymptomatic and early Huntington's disease: Selective white matter pathology and its relationship to clinical measures","volume":"21","author":"Rosas","year":"2006","journal-title":"Mov. Disord."},{"key":"10.1016\/j.omtn.2025.102710_bib123","first-page":"2621","article-title":"Structural and molecular myelination deficits occur prior to neuronal loss in the YAC128 and BACHD models of Huntington disease","volume":"25","author":"Teo","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib124","doi-asserted-by":"crossref","first-page":"4464","DOI":"10.1007\/s12035-018-1393-1","article-title":"Laquinimod Treatment Improves Myelination Deficits at the Transcriptional and Ultrastructural Levels in the YAC128 Mouse Model of Huntington Disease","volume":"56","author":"Garcia-Miralles","year":"2019","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.omtn.2025.102710_bib125","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.jconrel.2024.01.011","article-title":"Systemic delivery of mutant huntingtin lowering antisense oligonucleotides to the brain using apolipoprotein A-I nanodisks for Huntington disease","volume":"367","author":"Caron","year":"2024","journal-title":"J Control Release"},{"key":"10.1016\/j.omtn.2025.102710_bib126","doi-asserted-by":"crossref","first-page":"2093","DOI":"10.1038\/mt.2014.153","article-title":"In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides","volume":"22","author":"Southwell","year":"2014","journal-title":"Mol. Ther."},{"key":"10.1016\/j.omtn.2025.102710_bib127","doi-asserted-by":"crossref","first-page":"7109","DOI":"10.1093\/nar\/gkad371","article-title":"A single-cell map of antisense oligonucleotide activity in the brain","volume":"51","author":"Mortberg","year":"2023","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.omtn.2025.102710_bib128","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.aaw8546","article-title":"Astrocyte molecular signatures in Huntington's disease","volume":"11","author":"Diaz-Castro","year":"2019","journal-title":"Sci. Transl. Med."},{"key":"10.1016\/j.omtn.2025.102710_bib129","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1002\/ana.410370213","article-title":"Huntington's disease gene: regional and cellular expression in brain of normal and affected individuals","volume":"37","author":"Landwehrmeyer","year":"1995","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib130","doi-asserted-by":"crossref","DOI":"10.1016\/j.celrep.2022.111953","article-title":"Neuronal and astrocytic contributions to Huntington's disease dissected with zinc finger protein transcriptional repressors","volume":"42","author":"Gangwani","year":"2023","journal-title":"Cell Rep."},{"key":"10.1016\/j.omtn.2025.102710_bib131","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1093\/hmg\/ddl013","article-title":"Regional and cellular gene expression changes in human Huntington's disease brain","volume":"15","author":"Hodges","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib132","doi-asserted-by":"crossref","first-page":"1845","DOI":"10.1093\/hmg\/ddm133","article-title":"Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage","volume":"16","author":"Kuhn","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib133","doi-asserted-by":"crossref","first-page":"9544","DOI":"10.1523\/JNEUROSCI.1291-11.2011","article-title":"Peroxisome-proliferator-activated receptor gamma coactivator 1 alpha contributes to dysmyelination in experimental models of Huntington's disease","volume":"31","author":"Xiang","year":"2011","journal-title":"J. Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib134","doi-asserted-by":"crossref","DOI":"10.1172\/jci.insight.161769","article-title":"Benefits of global mutant huntingtin lowering diminish over time in a Huntington's disease mouse model","volume":"7","author":"Marchionini","year":"2022","journal-title":"JCI Insight"},{"key":"10.1016\/j.omtn.2025.102710_bib135","doi-asserted-by":"crossref","first-page":"1259","DOI":"10.1093\/hmg\/9.9.1259","article-title":"Decreased expression of striatal signaling genes in a mouse model of Huntington's disease","volume":"9","author":"Luthi-Carter","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib136","doi-asserted-by":"crossref","first-page":"1173","DOI":"10.1016\/j.neuron.2022.01.006","article-title":"Uninterrupted CAG repeat drives striatum-selective transcriptionopathy and nuclear pathogenesis in human Huntingtin BAC mice","volume":"110","author":"Gu","year":"2022","journal-title":"Neuron"},{"key":"10.1016\/j.omtn.2025.102710_bib137","doi-asserted-by":"crossref","first-page":"1438","DOI":"10.1093\/hmg\/ddq018","article-title":"Transcriptional changes in Huntington disease identified using genome-wide expression profiling and cross-platform analysis","volume":"19","author":"Becanovic","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib138","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1093\/hmg\/ddp527","article-title":"Expression analysis of novel striatal-enriched genes in Huntington disease","volume":"19","author":"Mazarei","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtn.2025.102710_bib139","doi-asserted-by":"crossref","first-page":"967","DOI":"10.1016\/j.neuroscience.2003.11.009","article-title":"Striatal phosphodiesterase mRNA and protein levels are reduced in Huntington's disease transgenic mice prior to the onset of motor symptoms","volume":"123","author":"Hebb","year":"2004","journal-title":"Neuroscience"},{"key":"10.1016\/j.omtn.2025.102710_bib140","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1002\/ana.410370309","article-title":"Reduced expression of preproenkephalin in striatal neurons from Huntington's disease patients","volume":"37","author":"Richfield","year":"1995","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib141","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1002\/ana.410420213","article-title":"Dopamine D1 and D2 receptor gene expression in the striatum in Huntington's disease","volume":"42","author":"Augood","year":"1997","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.omtn.2025.102710_bib142","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1016\/0306-4522(95)00595-1","article-title":"Reduction in enkephalin and substance P messenger RNA in the striatum of early grade Huntington's disease: a detailed cellular in situ hybridization study","volume":"72","author":"Augood","year":"1996","journal-title":"Neuroscience"},{"key":"10.1016\/j.omtn.2025.102710_bib143","doi-asserted-by":"crossref","first-page":"1212","DOI":"10.1016\/j.neuron.2015.02.026","article-title":"Mutant huntingtin downregulates myelin regulatory factor-mediated myelin gene expression and affects mature oligodendrocytes","volume":"85","author":"Huang","year":"2015","journal-title":"Neuron"},{"key":"10.1016\/j.omtn.2025.102710_bib144","doi-asserted-by":"crossref","first-page":"7791","DOI":"10.1038\/s41467-022-35388-x","article-title":"Huntington disease oligodendrocyte maturation deficits revealed by single-nucleus RNAseq are rescued by thiamine-biotin supplementation","volume":"13","author":"Lim","year":"2022","journal-title":"Nat. Commun."},{"key":"10.1016\/j.omtn.2025.102710_bib145","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1186\/s40478-024-01871-3","article-title":"Mapping the glial transcriptome in Huntington's disease using snRNAseq: selective disruption of glial signatures across brain regions","volume":"12","author":"Bostrand","year":"2024","journal-title":"Acta Neuropathol Commun"},{"key":"10.1016\/j.omtn.2025.102710_bib146","doi-asserted-by":"crossref","first-page":"2307","DOI":"10.1056\/NEJMoa1900907","article-title":"Targeting Huntingtin Expression in Patients with Huntington's Disease","volume":"380","author":"Tabrizi","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.omtn.2025.102710_bib147","doi-asserted-by":"crossref","first-page":"2203","DOI":"10.1056\/NEJMc2300400","article-title":"Tominersen in Adults with Manifest Huntington's Disease","volume":"389","author":"McColgan","year":"2023","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.omtn.2025.102710_bib148","doi-asserted-by":"crossref","first-page":"2366","DOI":"10.1073\/pnas.1221891110","article-title":"Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease","volume":"110","author":"Sathasivam","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.omtn.2025.102710_bib149","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1002\/mrm.10436","article-title":"Automatic segmentation of the brain and intracranial cerebrospinal fluid in T1-weighted volume MRI scans of the head, and its application to serial cerebral and intracranial volumetry","volume":"49","author":"Lemieux","year":"2003","journal-title":"Magn. Reson. Med."},{"key":"10.1016\/j.omtn.2025.102710_bib150","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1097\/01.sap.0000250653.77090.97","article-title":"Comparison of three methods for the estimation of total intracranial volume: stereologic, planimetric, and anthropometric approaches","volume":"58","author":"Acer","year":"2007","journal-title":"Ann. Plast. Surg."},{"key":"10.1016\/j.omtn.2025.102710_bib151","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1186\/s13195-021-00793-9","article-title":"Development of a novel, sensitive translational immunoassay to detect plasma glial fibrillary acidic protein (GFAP) after murine traumatic brain injury","volume":"13","author":"Button","year":"2021","journal-title":"Alzheimers Res. Ther."},{"key":"10.1016\/j.omtn.2025.102710_bib152","article-title":"Automatic structural parcellation of mouse brain MRI using multi-atlas label fusion","volume":"9","author":"Ma","year":"2014","journal-title":"PLoS One"},{"key":"10.1016\/j.omtn.2025.102710_bib153","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3389\/neuro.05.001.2008","article-title":"Vivo 3D Digital Atlas Database of the Adult C57BL\/6J Mouse Brain by Magnetic Resonance Microscopy","volume":"2","author":"Ma","year":"2008","journal-title":"Front. Neuroanat."},{"key":"10.1016\/j.omtn.2025.102710_bib154","doi-asserted-by":"crossref","DOI":"10.1117\/1.JMI.1.2.024003","article-title":"Global image registration using a symmetric block-matching approach","volume":"1","author":"Modat","year":"2014","journal-title":"J. Med. Imaging"},{"key":"10.1016\/j.omtn.2025.102710_bib155","series-title":"Medical Image Computing and Computer-Assisted Intervention \u2013 MICCAI 2000","article-title":"Block Matching: A General Framework to Improve Robustness of Rigid Registration of Medical Images","author":"Ourselin","year":"2000"},{"key":"10.1016\/j.omtn.2025.102710_bib156","series-title":"Biomedical Image Registration","article-title":"Inverse-Consistent Symmetric Free Form Deformation","author":"Modat","year":"2012"},{"key":"10.1016\/j.omtn.2025.102710_bib157","series-title":"2012 IEEE Workshop on Mathematical Methods in Biomedical Image Analysis","article-title":"Parametric non-rigid registration using a stationary velocity field","author":"Modat","year":"2012"},{"key":"10.1016\/j.omtn.2025.102710_bib158","doi-asserted-by":"crossref","first-page":"712","DOI":"10.1109\/42.796284","article-title":"Nonrigid registration using free-form deformations: application to breast MR images","volume":"18","author":"Rueckert","year":"1999","journal-title":"IEEE Trans. Med. Imaging"},{"key":"10.1016\/j.omtn.2025.102710_bib159","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1016\/j.media.2013.02.006","article-title":"STEPS: Similarity and Truth Estimation for Propagated Segmentations and its application to hippocampal segmentation and brain parcelation","volume":"17","author":"Jorge Cardoso","year":"2013","journal-title":"Med. Image Anal."},{"key":"10.1016\/j.omtn.2025.102710_bib160","doi-asserted-by":"crossref","first-page":"355","DOI":"10.3233\/JHD-200463","article-title":"Reliable Resolution of Full-Length Huntingtin Alleles by Quantitative Immunoblotting","volume":"10","author":"Caron","year":"2021","journal-title":"J. Huntingtons Dis."},{"key":"10.1016\/j.omtn.2025.102710_bib161","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1186\/s13059-014-0550-8","article-title":"Moderated estimation of fold change and dispersion for RNA-seq data with DESeq2","volume":"15","author":"Love","year":"2014","journal-title":"Genome Biol."},{"key":"10.1016\/j.omtn.2025.102710_bib162","doi-asserted-by":"crossref","first-page":"W193","DOI":"10.1093\/nar\/gkm226","article-title":"g:Profiler--a web-based toolset for functional profiling of gene lists from large-scale experiments","volume":"35","author":"Reimand","year":"2007","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.omtn.2025.102710_bib163","doi-asserted-by":"crossref","first-page":"W207","DOI":"10.1093\/nar\/gkad347","article-title":"g:Profiler-interoperable web service for functional enrichment analysis and gene identifier mapping (2023 update)","volume":"51","author":"Kolberg","year":"2023","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.omtn.2025.102710_bib164","doi-asserted-by":"crossref","first-page":"4218","DOI":"10.1093\/nar\/gkq130","article-title":"Analytical approaches to RNA profiling data for the identification of genes enriched in specific cells","volume":"38","author":"Dougherty","year":"2010","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.omtn.2025.102710_bib165","doi-asserted-by":"crossref","first-page":"1420","DOI":"10.1523\/JNEUROSCI.4488-13.2014","article-title":"Cell type-specific expression analysis to identify putative cellular mechanisms for neurogenetic disorders","volume":"34","author":"Xu","year":"2014","journal-title":"J. Neurosci."},{"key":"10.1016\/j.omtn.2025.102710_bib166","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1186\/1471-2105-14-128","article-title":"Enrichr: interactive and collaborative HTML5 gene list enrichment analysis tool","volume":"14","author":"Chen","year":"2013","journal-title":"BMC Bioinf."},{"key":"10.1016\/j.omtn.2025.102710_bib167","doi-asserted-by":"crossref","first-page":"W90","DOI":"10.1093\/nar\/gkw377","article-title":"Enrichr: a comprehensive gene set enrichment analysis web server 2016 update","volume":"44","author":"Kuleshov","year":"2016","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.omtn.2025.102710_bib168","doi-asserted-by":"crossref","first-page":"1524","DOI":"10.1016\/j.cell.2025.01.031","article-title":"Distinct mismatch-repair complex genes set neuronal CAG-repeat expansion rate to drive selective pathogenesis in HD mice","volume":"188","author":"Wang","year":"2025","journal-title":"Cell"}],"container-title":["Molecular Therapy Nucleic Acids"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2162253125002641?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2162253125002641?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T16:18:05Z","timestamp":1773159485000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2162253125002641"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,12]]},"references-count":164,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2025,12]]}},"alternative-id":["S2162253125002641"],"URL":"https:\/\/doi.org\/10.1016\/j.omtn.2025.102710","relation":{},"ISSN":["2162-2531"],"issn-type":[{"value":"2162-2531","type":"print"}],"subject":[],"published":{"date-parts":[[2025,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Molecular and imaging biomarker responses to brain mutant HTT lowering in a mouse model of Huntington disease","name":"articletitle","label":"Article Title"},{"value":"Molecular Therapy Nucleic Acids","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.omtn.2025.102710","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 The Authors. Published by Elsevier Inc. on behalf of The American Society of Gene and Cell Therapy.","name":"copyright","label":"Copyright"}],"article-number":"102710"}}