{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T19:51:57Z","timestamp":1792093917094,"version":"4.4.0"},"reference-count":166,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T00:00:00Z","timestamp":1791676800000},"content-version":"vor","delay-in-days":10,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecules and Cells"],"published-print":{"date-parts":[[2026,10]]},"DOI":"10.1016\/j.mocell.2026.100406","type":"journal-article","created":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T15:53:59Z","timestamp":1791993239000},"page":"100406","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":0,"title":["Lipid dysregulation in polyglutamine diseases"],"prefix":"10.1016","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-3043-3002","authenticated-orcid":false,"given":"Luhan","family":"Ni","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vaishnavi","family":"Veeranki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-2001-9243","authenticated-orcid":false,"given":"Byron","family":"Huang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5331-210X","authenticated-orcid":false,"given":"Janghoo","family":"Lim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.mocell.2026.100406_bib1","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1007\/978-1-4020-8831-5_9","article-title":"Altered lipid metabolism in brain injury and disorders","author":"Adibhatla","year":"2008","journal-title":"Subcell Biochem 49"},{"key":"10.1016\/j.mocell.2026.100406_bib2","doi-asserted-by":"crossref","DOI":"10.1038\/srep31411","article-title":"Altered lipid metabolism in Drosophila model of Huntington's disease","volume":"6","author":"Aditi","year":"2016","journal-title":"Sci Rep"},{"key":"10.1016\/j.mocell.2026.100406_bib3","doi-asserted-by":"crossref","first-page":"229","DOI":"10.2217\/17460875.2.2.229","article-title":"Triacylglycerol metabolism in adipose tissue","volume":"2","author":"Ahmadian","year":"2007","journal-title":"Future Lipidol"},{"key":"10.1016\/j.mocell.2026.100406_bib4","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1186\/s40478-020-0880-6","article-title":"Single-nucleus RNA-seq identifies Huntington disease astrocyte states","volume":"8","author":"Al-Dalahmah","year":"2020","journal-title":"Acta Neuropathol Commun"},{"key":"10.1016\/j.mocell.2026.100406_bib5","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1016\/j.tibs.2011.06.005","article-title":"Shaping up the membrane: diacylglycerol coordinates spatial orientation of signaling","volume":"36","author":"Almena","year":"2011","journal-title":"Trends Biochem Sci"},{"key":"10.1016\/j.mocell.2026.100406_bib6","doi-asserted-by":"crossref","first-page":"1537","DOI":"10.15252\/emmm.201707763","article-title":"Disease-modifying effects of ganglioside GM1 in Huntington's disease models","volume":"9","author":"Alpaugh","year":"2017","journal-title":"EMBO Mol Med"},{"key":"10.1016\/j.mocell.2026.100406_bib7","doi-asserted-by":"crossref","first-page":"3626","DOI":"10.1016\/j.cub.2017.10.054","article-title":"Stall in Canonical Autophagy-Lysosome Pathways Prompts Nucleophagy-Based Nuclear Breakdown in Neurodegeneration","volume":"27","author":"Baron","year":"2017","journal-title":"Curr Biol"},{"key":"10.1016\/j.mocell.2026.100406_bib8","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.adn5449","article-title":"Dysregulation of zebrin-II cell subtypes in the cerebellum is a shared feature across polyglutamine ataxia mouse models and patients","volume":"16","author":"Bartelt","year":"2024","journal-title":"Sci Transl Med"},{"key":"10.1016\/j.mocell.2026.100406_bib9","doi-asserted-by":"crossref","first-page":"S91","DOI":"10.1194\/jlr.R800080-JLR200","article-title":"Bioactive sphingolipids: metabolism and function","volume":"50","author":"Bartke","year":"2009","journal-title":"J Lipid Res"},{"key":"10.1016\/j.mocell.2026.100406_bib10","doi-asserted-by":"crossref","DOI":"10.3390\/nu13062061","article-title":"Maternal Supply of Both Arachidonic and Docosahexaenoic Acids Is Required for Optimal Neurodevelopment","volume":"13","author":"Basak","year":"2021","journal-title":"Nutrients"},{"key":"10.1016\/j.mocell.2026.100406_bib11","doi-asserted-by":"crossref","first-page":"1580","DOI":"10.1001\/archneurol.2011.636","article-title":"The APOE epsilon2 allele increases the risk of earlier age at onset in Machado-Joseph disease","volume":"68","author":"Bettencourt","year":"2011","journal-title":"Arch Neurol"},{"key":"10.1016\/j.mocell.2026.100406_bib12","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1007\/978-3-031-12390-0_2","article-title":"Cells of the Central Nervous System: An Overview of Their Structure and Function","volume":"29","author":"Bigbee","year":"2023","journal-title":"Adv Neurobiol"},{"key":"10.1016\/j.mocell.2026.100406_bib13","doi-asserted-by":"crossref","first-page":"3175","DOI":"10.1093\/brain\/awab186","article-title":"SREBP2 gene therapy targeting striatal astrocytes ameliorates Huntington's disease phenotypes","volume":"144","author":"Birolini","year":"2021","journal-title":"Brain"},{"key":"10.1016\/j.mocell.2026.100406_bib14","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.2016969118","article-title":"Sphingolipid metabolism governs Purkinje cell patterned degeneration in Atxn1[82Q]\/+ mice","volume":"118","author":"Blot","year":"2021","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.mocell.2026.100406_bib15","doi-asserted-by":"crossref","first-page":"1953","DOI":"10.1016\/j.bbamem.2013.04.025","article-title":"Huntingtin disrupts lipid bilayers in a polyQ-length dependent manner","volume":"1828","author":"Burke","year":"2013","journal-title":"Biochim Biophys Acta"},{"key":"10.1016\/j.mocell.2026.100406_bib16","doi-asserted-by":"crossref","DOI":"10.1016\/j.plipres.2023.101224","article-title":"Sphingolipids and impaired hypoxic stress responses in Huntington disease","volume":"90","author":"Burtscher","year":"2023","journal-title":"Prog Lipid Res"},{"key":"10.1016\/j.mocell.2026.100406_bib17","doi-asserted-by":"crossref","DOI":"10.1016\/j.nbd.2021.105289","article-title":"Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression","volume":"152","author":"Canet-Pons","year":"2021","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib18","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0134465","article-title":"HdhQ111 Mice Exhibit Tissue Specific Metabolite Profiles that Include Striatal Lipid Accumulation","volume":"10","author":"Carroll","year":"2015","journal-title":"PLoS One"},{"key":"10.1016\/j.mocell.2026.100406_bib19","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1038\/s41593-023-01288-6","article-title":"Lipid-accumulated reactive astrocytes promote disease progression in epilepsy","volume":"26","author":"Chen","year":"2023","journal-title":"Nat Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib20","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/j.neuroscience.2015.01.003","article-title":"Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1","volume":"289","author":"Cvetanovic","year":"2015","journal-title":"Neuroscience"},{"key":"10.1016\/j.mocell.2026.100406_bib21","doi-asserted-by":"crossref","DOI":"10.3390\/molecules23102583","article-title":"The Various Roles of Fatty Acids","volume":"23","author":"de Carvalho","year":"2018","journal-title":"Molecules"},{"key":"10.1016\/j.mocell.2026.100406_bib22","first-page":"3893","article-title":"A comprehensive endocrine description of Kennedy's disease revealing androgen insensitivity linked to CAG repeat length","volume":"87","author":"Dejager","year":"2002","journal-title":"J Clin Endocrinol Metab"},{"key":"10.1016\/j.mocell.2026.100406_bib23","doi-asserted-by":"crossref","first-page":"748","DOI":"10.1111\/j.1471-4159.2010.06964.x","article-title":"Cerebellar lipid differences between R6\/1 transgenic mice and humans with Huntington's disease","volume":"115","author":"Denny","year":"2010","journal-title":"J Neurochem"},{"key":"10.1016\/j.mocell.2026.100406_bib24","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/j.nbd.2007.05.003","article-title":"Glycolipid and ganglioside metabolism imbalances in Huntington's disease","volume":"27","author":"Desplats","year":"2007","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib25","doi-asserted-by":"crossref","first-page":"5280","DOI":"10.1038\/s41598-017-05709-y","article-title":"Defective Sphingosine-1-phosphate metabolism is a druggable target in Huntington's disease","volume":"7","author":"Di Pardo","year":"2017","journal-title":"Sci Rep"},{"key":"10.1016\/j.mocell.2026.100406_bib26","doi-asserted-by":"crossref","first-page":"2251","DOI":"10.1093\/hmg\/ddt615","article-title":"FTY720 (fingolimod) is a neuroprotective and disease-modifying agent in cellular and mouse models of Huntington disease","volume":"23","author":"Di Pardo","year":"2014","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.mocell.2026.100406_bib27","doi-asserted-by":"crossref","first-page":"3528","DOI":"10.1073\/pnas.1114502109","article-title":"Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice","volume":"109","author":"Di Pardo","year":"2012","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.mocell.2026.100406_bib28","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1042\/BCJ20180022","article-title":"Understanding phosphoinositides: rare, dynamic, and essential membrane phospholipids","volume":"476","author":"Dickson","year":"2019","journal-title":"Biochem J"},{"key":"10.1016\/j.mocell.2026.100406_bib29","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.1194\/jlr.R400004-JLR200","article-title":"Thematic review series: brain Lipids. Cholesterol metabolism in the central nervous system during early development and in the mature animal","volume":"45","author":"Dietschy","year":"2004","journal-title":"J Lipid Res"},{"key":"10.1016\/j.mocell.2026.100406_bib30","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1007\/s12035-014-8663-3","article-title":"Mitochondrial membrane fluidity is consistently increased in different models of Huntington disease: restorative effects of olesoxime","volume":"50","author":"Eckmann","year":"2014","journal-title":"Mol Neurobiol"},{"key":"10.1016\/j.mocell.2026.100406_bib31","doi-asserted-by":"crossref","first-page":"441","DOI":"10.3727\/096368914X678454","article-title":"Polyglutamine (PolyQ) diseases: genetics to treatments","volume":"23","author":"Fan","year":"2014","journal-title":"Cell Transplant"},{"key":"10.1016\/j.mocell.2026.100406_bib32","doi-asserted-by":"crossref","first-page":"742","DOI":"10.3389\/fnins.2020.00742","article-title":"Lipid Droplets in Neurodegenerative Disorders","volume":"14","author":"Farmer","year":"2020","journal-title":"Front Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib33","doi-asserted-by":"crossref","DOI":"10.1016\/j.nbd.2022.105933","article-title":"Longitudinal spatial mapping of lipid metabolites reveals pre-symptomatic changes in the hippocampi of Huntington's disease transgenic mice","volume":"176","author":"Farzana","year":"2023","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib34","doi-asserted-by":"crossref","first-page":"1017","DOI":"10.1152\/physrev.00004.2003","article-title":"Role of endogenous cannabinoids in synaptic signaling","volume":"83","author":"Freund","year":"2003","journal-title":"Physiol Rev"},{"key":"10.1016\/j.mocell.2026.100406_bib35","doi-asserted-by":"crossref","first-page":"1350","DOI":"10.1038\/s41593-018-0221-2","article-title":"Selective vulnerability in neurodegenerative diseases","volume":"21","author":"Fu","year":"2018","journal-title":"Nat Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib36","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/bs.pmbts.2018.01.009","article-title":"Gangliosides in Inflammation and Neurodegeneration","volume":"156","author":"Furukawa","year":"2018","journal-title":"Prog Mol Biol Transl Sci"},{"key":"10.1016\/j.mocell.2026.100406_bib37","doi-asserted-by":"crossref","first-page":"1551","DOI":"10.1046\/j.1471-4159.1995.65041551.x","article-title":"Sphingolipid Biosynthesis Is Necessary for Dendrite Growth and Survival of Cerebellar Purkinje-Cells in Culture","volume":"65","author":"Furuya","year":"1995","journal-title":"Journal of Neurochemistry"},{"key":"10.1016\/j.mocell.2026.100406_bib38","doi-asserted-by":"crossref","DOI":"10.1038\/s41586-026-11050-0","article-title":"The lipidomic architecture of the mouse brain","author":"Fusar Bassini","year":"2026","journal-title":"Nature"},{"key":"10.1016\/j.mocell.2026.100406_bib39","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.mocell.2026.100406_bib40","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1038\/nrg1691","article-title":"Diseases of unstable repeat expansion: mechanisms and common principles","volume":"6","author":"Gatchel","year":"2005","journal-title":"Nat Rev Genet"},{"key":"10.1016\/j.mocell.2026.100406_bib41","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1111\/jnc.15133","article-title":"The role of Sphingolipids in myelination and myelin stability and their involvement in childhood and adult demyelinating disorders","volume":"156","author":"Giussani","year":"2021","journal-title":"J Neurochem"},{"key":"10.1016\/j.mocell.2026.100406_bib42","doi-asserted-by":"crossref","first-page":"2430","DOI":"10.1016\/j.bbadis.2018.04.012","article-title":"Targeted biochemical profiling of brain from Huntington's disease patients reveals novel metabolic pathways of interest","volume":"1864","author":"Graham","year":"2018","journal-title":"Biochim Biophys Acta Mol Basis Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib43","doi-asserted-by":"crossref","first-page":"2481","DOI":"10.1212\/WNL.0000000000004748","article-title":"Nonalcoholic fatty liver disease in spinal and bulbar muscular atrophy","volume":"89","author":"Guber","year":"2017","journal-title":"Neurology"},{"key":"10.1016\/j.mocell.2026.100406_bib44","doi-asserted-by":"crossref","DOI":"10.1101\/cshperspect.a004762","article-title":"Distribution and functions of sterols and sphingolipids","volume":"3","author":"Hannich","year":"2011","journal-title":"Cold Spring Harb Perspect Biol"},{"key":"10.1016\/j.mocell.2026.100406_bib45","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1038\/nrm2329","article-title":"Principles of bioactive lipid signalling: lessons from sphingolipids","volume":"9","author":"Hannun","year":"2008","journal-title":"Nat Rev Mol Cell Biol"},{"key":"10.1016\/j.mocell.2026.100406_bib46","doi-asserted-by":"crossref","first-page":"27855","DOI":"10.1074\/jbc.R111.254359","article-title":"Many ceramides","volume":"286","author":"Hannun","year":"2011","journal-title":"J Biol Chem"},{"key":"10.1016\/j.mocell.2026.100406_bib47","doi-asserted-by":"crossref","first-page":"2807","DOI":"10.1002\/2211-5463.13285","article-title":"Multiplatform metabolomic analysis of the R6\/2 mouse model of Huntington's disease","volume":"11","author":"Hashimoto","year":"2021","journal-title":"Febs Open Bio"},{"key":"10.1016\/j.mocell.2026.100406_bib48","doi-asserted-by":"crossref","first-page":"799","DOI":"10.1194\/jlr.R046094","article-title":"Diversity and function of membrane glycerophospholipids generated by the remodeling pathway in mammalian cells","volume":"55","author":"Hishikawa","year":"2014","journal-title":"J Lipid Res"},{"key":"10.1016\/j.mocell.2026.100406_bib49","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1111\/j.1601-183X.2007.00350.x","article-title":"Brain gene expression correlates with changes in behavior in the R6\/1 mouse model of Huntington's disease","volume":"7","author":"Hodges","year":"2008","journal-title":"Genes Brain Behav"},{"key":"10.1016\/j.mocell.2026.100406_bib50","doi-asserted-by":"crossref","DOI":"10.3389\/fcell.2021.661486","article-title":"Glial Cells Promote Myelin Formation and Elimination","volume":"9","author":"Hughes","year":"2021","journal-title":"Front Cell Dev Biol"},{"key":"10.1016\/j.mocell.2026.100406_bib51","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1111\/jnc.14468","article-title":"Subventricular zone lipidomic architecture loss in Huntington's disease","volume":"146","author":"Hunter","year":"2018","journal-title":"J Neurochem"},{"key":"10.1016\/j.mocell.2026.100406_bib52","doi-asserted-by":"crossref","first-page":"2158","DOI":"10.1111\/jnc.15325","article-title":"An imaging mass spectrometry atlas of lipids in the human neurologically normal and Huntington's disease caudate nucleus","volume":"157","author":"Hunter","year":"2021","journal-title":"J Neurochem"},{"key":"10.1016\/j.mocell.2026.100406_bib53","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1186\/s12944-019-0965-z","article-title":"Role of cholesterol and sphingolipids in brain development and neurological diseases","volume":"18","author":"Hussain","year":"2019","journal-title":"Lipids Health Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib54","doi-asserted-by":"crossref","DOI":"10.3389\/fnsyn.2021.618391","article-title":"Disposition of Proteins and Lipids in Synaptic Membrane Compartments Is Altered in Q175\/Q7 Huntington's Disease Mouse Striatum","volume":"13","author":"Iuliano","year":"2021","journal-title":"Front Synaptic Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib55","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.plipres.2013.10.002","article-title":"Long-chain polyunsaturated fatty acids (LCPUFA) from genesis to senescence: the influence of LCPUFA on neural development, aging, and neurodegeneration","volume":"53","author":"Janssen","year":"2014","journal-title":"Prog Lipid Res"},{"key":"10.1016\/j.mocell.2026.100406_bib56","doi-asserted-by":"crossref","DOI":"10.1002\/jms.5008","article-title":"Spatial neurolipidomics-MALDI mass spectrometry imaging of lipids in brain pathologies","volume":"59","author":"Jha","year":"2024","journal-title":"J Mass Spectrom"},{"key":"10.1016\/j.mocell.2026.100406_bib57","doi-asserted-by":"crossref","first-page":"2508","DOI":"10.1093\/hmg\/ddv016","article-title":"Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease","volume":"24","author":"Jin","year":"2015","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.mocell.2026.100406_bib58","doi-asserted-by":"crossref","DOI":"10.1016\/j.biopha.2025.117827","article-title":"The role of mitochondrial dysfunction in Huntington's disease: Implications for therapeutic targeting","volume":"183","author":"Joshi","year":"2025","journal-title":"Biomed Pharmacother"},{"key":"10.1016\/j.mocell.2026.100406_bib59","doi-asserted-by":"crossref","DOI":"10.3389\/fnagi.2022.797220","article-title":"Altered Cholesterol Homeostasis in Huntington's Disease","volume":"14","author":"Kacher","year":"2022","journal-title":"Front Aging Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib60","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s100480050046","article-title":"Dentatorubral-pallidoluysian atrophy or Naito-Oyanagi disease","volume":"2","author":"Kanazawa","year":"1998","journal-title":"Neurogenetics"},{"key":"10.1016\/j.mocell.2026.100406_bib61","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/ng1194-221","article-title":"CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1","volume":"8","author":"Kawaguchi","year":"1994","journal-title":"Nat Genet"},{"key":"10.1016\/j.mocell.2026.100406_bib62","doi-asserted-by":"crossref","first-page":"1585","DOI":"10.1111\/j.1471-4159.2009.06255.x","article-title":"Polyglutamine expansion in huntingtin alters its interaction with phospholipids","volume":"110","author":"Kegel","year":"2009","journal-title":"J Neurochem"},{"key":"10.1016\/j.mocell.2026.100406_bib63","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1016\/j.bbrc.2009.07.039","article-title":"Polyglutamine expansion in huntingtin increases its insertion into lipid bilayers","volume":"387","author":"Kegel","year":"2009","journal-title":"Biochem Biophys Res Commun"},{"key":"10.1016\/j.mocell.2026.100406_bib64","first-page":"671","article-title":"Progressive proximal spinal and bulbar muscular atrophy of late onset","volume":"18","author":"Kennedy","year":"1968","journal-title":"A sex-linked recessive trait. Neurology"},{"key":"10.1016\/j.mocell.2026.100406_bib65","first-page":"35215","article-title":"Inhibition of neuronal apoptosis by docosahexaenoic acid (22:6n-3)","volume":"275","author":"Kim","year":"2000","journal-title":"Role of phosphatidylserine in antiapoptotic effect. J Biol Chem"},{"key":"10.1016\/j.mocell.2026.100406_bib66","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.plipres.2014.06.002","article-title":"Phosphatidylserine in the brain: metabolism and function","volume":"56","author":"Kim","year":"2014","journal-title":"Prog Lipid Res"},{"key":"10.1016\/j.mocell.2026.100406_bib67","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1001\/archneur.58.2.273","article-title":"Progression of symptoms in the early and middle stages of Huntington disease","volume":"58","author":"Kirkwood","year":"2001","journal-title":"Arch Neurol"},{"key":"10.1016\/j.mocell.2026.100406_bib68","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1038\/s41572-019-0074-3","article-title":"Spinocerebellar ataxia","volume":"5","author":"Klockgether","year":"2019","journal-title":"Nat Rev Dis Primers"},{"key":"10.1016\/j.mocell.2026.100406_bib69","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/j.jns.2004.11.036","article-title":"Cholesterol homeostasis failure as a unifying cause of synaptic degeneration","volume":"229-230","author":"Koudinov","year":"2005","journal-title":"J Neurol Sci"},{"key":"10.1016\/j.mocell.2026.100406_bib70","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1038\/s43587-023-00368-3","article-title":"Neuronal APOE4 removal protects against tau-mediated gliosis, neurodegeneration and myelin deficits","volume":"3","author":"Koutsodendris","year":"2023","journal-title":"Nat Aging"},{"key":"10.1016\/j.mocell.2026.100406_bib71","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1111\/nan.12286","article-title":"Evidence for altered cholesterol metabolism in Huntington's disease post mortem brain tissue","volume":"42","author":"Kreilaus","year":"2016","journal-title":"Neuropath Appl Neuro"},{"key":"10.1016\/j.mocell.2026.100406_bib72","doi-asserted-by":"crossref","DOI":"10.3389\/fcell.2021.732820","article-title":"The \"Neuro-Glial-Vascular\" Unit: The Role of Glia in Neurovascular Unit Formation and Dysfunction","volume":"9","author":"Kugler","year":"2021","journal-title":"Front Cell Dev Biol"},{"key":"10.1016\/j.mocell.2026.100406_bib73","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1016\/S0896-6273(03)00328-3","article-title":"Polyglutamines placed into context","volume":"38","author":"La Spada","year":"2003","journal-title":"Neuron"},{"key":"10.1016\/j.mocell.2026.100406_bib74","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1038\/352077a0","article-title":"Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy","volume":"352","author":"Spada","year":"1991","journal-title":"Nature"},{"key":"10.1016\/j.mocell.2026.100406_bib75","doi-asserted-by":"crossref","first-page":"1976","DOI":"10.1002\/mds.23176","article-title":"Impaired insulin sensitivity and secretion in normoglycemic patients with spinocerebellar ataxia type 1","volume":"25","author":"Lalic","year":"2010","journal-title":"Mov Disord"},{"key":"10.1016\/j.mocell.2026.100406_bib76","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/j.semcdb.2020.07.017","article-title":"Lipid metabolism in astrocytic structure and function","volume":"112","author":"Lee","year":"2021","journal-title":"Semin Cell Dev Biol"},{"key":"10.1016\/j.mocell.2026.100406_bib77","doi-asserted-by":"crossref","DOI":"10.1016\/j.phrs.2026.108375","article-title":"Cholesterol restores corticostriatal synaptic connectivity in Huntington's disease through BDNF\/TrkB signalling, supporting a role for cholesterol pathways implicated by human genetic modifier studies","volume":"231","author":"Lenci","year":"2026","journal-title":"Pharmacol Res"},{"key":"10.1016\/j.mocell.2026.100406_bib78","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.nbd.2013.03.013","article-title":"Plasma 24S-hydroxycholesterol correlation with markers of Huntington disease progression","volume":"55","author":"Leoni","year":"2013","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib79","doi-asserted-by":"crossref","first-page":"2851","DOI":"10.1093\/brain\/awn212","article-title":"Plasma 24S-hydroxycholesterol and caudate MRI in pre-manifest and early Huntingtons disease","volume":"131","author":"Leoni","year":"2008","journal-title":"Brain"},{"key":"10.1016\/j.mocell.2026.100406_bib80","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1146\/annurev-pathmechdis-012418-012857","article-title":"Polyglutamine Repeats in Neurodegenerative Diseases","volume":"14","author":"Lieberman","year":"2019","journal-title":"Annu Rev Pathol"},{"key":"10.1016\/j.mocell.2026.100406_bib81","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.mocell.2026.100406_bib82","doi-asserted-by":"crossref","first-page":"3454","DOI":"10.1093\/hmg\/ddw187","article-title":"Transcriptome sequencing reveals aberrant alternative splicing in Huntington's disease","volume":"25","author":"Lin","year":"2016","journal-title":"Human Molecular Genetics"},{"key":"10.1016\/j.mocell.2026.100406_bib83","article-title":"Transcriptomic and Metabolic Network Analysis of Metabolic Reprogramming and IGF-1 Modulation in SCA3 Transgenic Mice","volume":"22","author":"Lin","year":"2021","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.mocell.2026.100406_bib84","doi-asserted-by":"crossref","first-page":"248","DOI":"10.3389\/fncel.2016.00248","article-title":"Transient Developmental Purkinje Cell Axonal Torpedoes in Healthy and Ataxic Mouse Cerebellum","volume":"10","author":"Ljungberg","year":"2016","journal-title":"Front Cell Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib85","doi-asserted-by":"crossref","first-page":"393","DOI":"10.3389\/fphys.2020.00393","article-title":"Lipid and Lipoprotein Metabolism in Microglia","volume":"11","author":"Loving","year":"2020","journal-title":"Front Physiol"},{"key":"10.1016\/j.mocell.2026.100406_bib86","doi-asserted-by":"crossref","first-page":"7238","DOI":"10.1073\/pnas.96.13.7238","article-title":"cDNA cloning of cholesterol 24-hydroxylase, a mediator of cholesterol homeostasis in the brain","volume":"96","author":"Lund","year":"1999","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.mocell.2026.100406_bib87","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 Huntingtons-Disease Chromosomes","volume":"72","author":"Macdonald","year":"1993","journal-title":"Cell"},{"key":"10.1016\/j.mocell.2026.100406_bib88","doi-asserted-by":"crossref","first-page":"4072","DOI":"10.1523\/JNEUROSCI.6348-09.2010","article-title":"Impaired ganglioside metabolism in Huntington's disease and neuroprotective role of GM1","volume":"30","author":"Maglione","year":"2010","journal-title":"J Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib89","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1016\/j.ymgme.2007.10.002","article-title":"Low plasma total cholesterol in patients with Huntington's disease and first-degree relatives","volume":"93","author":"Markianos","year":"2008","journal-title":"Mol Genet Metab"},{"key":"10.1016\/j.mocell.2026.100406_bib90","article-title":"Cross-sectional analysis of plasma and CSF metabolomic markers in Huntington's disease for participants of varying functional disability: a pilot study","volume":"10","author":"McGarry","year":"2020","journal-title":"Sci Rep-Uk"},{"key":"10.1016\/j.mocell.2026.100406_bib91","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1007\/s00018-012-1038-x","article-title":"Ceramide function in the brain: when a slight tilt is enough","volume":"70","author":"Mencarelli","year":"2013","journal-title":"Cell Mol Life Sci"},{"key":"10.1016\/j.mocell.2026.100406_bib92","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1007\/s12275-017-6205-1","article-title":"The protein and neutral lipid composition of lipid droplets isolated from the fission yeast, Schizosaccharomyces pombe","volume":"55","author":"Meyers","year":"2017","journal-title":"J Microbiol"},{"key":"10.1016\/j.mocell.2026.100406_bib93","doi-asserted-by":"crossref","DOI":"10.1186\/s13023-016-0475-2","article-title":"Common disease signatures from gene expression analysis in Huntington's disease human blood and brain","volume":"11","author":"Mina","year":"2016","journal-title":"Orphanet J Rare Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib94","first-page":"68","article-title":"The fat brain","volume":"23","author":"Montesinos","year":"2020","journal-title":"Curr Opin Clin Nutr Metab Care"},{"key":"10.1016\/j.mocell.2026.100406_bib95","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1186\/s40478-020-01088-0","article-title":"Impaired lipid metabolism in astrocytes underlies degeneration of cortical projection neurons in hereditary spastic paraplegia","volume":"8","author":"Mou","year":"2020","journal-title":"Acta Neuropathol Commun"},{"key":"10.1016\/j.mocell.2026.100406_bib96","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1038\/ng0194-14","article-title":"Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p","volume":"6","author":"Nagafuchi","year":"1994","journal-title":"Nat Genet"},{"key":"10.1016\/j.mocell.2026.100406_bib97","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1007\/s00415-017-8405-3","article-title":"Correlation of insulin resistance and motor function in spinal and bulbar muscular atrophy","volume":"264","author":"Nakatsuji","year":"2017","journal-title":"J Neurol"},{"key":"10.1016\/j.mocell.2026.100406_bib98","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.devcel.2017.06.003","article-title":"DGAT1-Dependent Lipid Droplet Biogenesis Protects Mitochondrial Function during Starvation-Induced Autophagy","volume":"42","author":"Nguyen","year":"2017","journal-title":"Dev Cell"},{"key":"10.1016\/j.mocell.2026.100406_bib99","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0122279","article-title":"Genotype-phenotype correlation in Chinese patients with spinal and bulbar muscular atrophy","volume":"10","author":"Ni","year":"2015","journal-title":"PLoS One"},{"key":"10.1016\/j.mocell.2026.100406_bib100","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1016\/j.jns.2016.02.018","article-title":"Liver function in Huntington's disease assessed by blood biochemical analyses in a clinical setting","volume":"362","author":"Nielsen","year":"2016","journal-title":"Journal of the Neurological Sciences"},{"key":"10.1016\/j.mocell.2026.100406_bib101","doi-asserted-by":"crossref","first-page":"1577","DOI":"10.1038\/cdd.2010.31","article-title":"Neurodegeneration by polyglutamine Atrophin is not rescued by induction of autophagy","volume":"17","author":"Nisoli","year":"2010","journal-title":"Cell Death Differ"},{"key":"10.1016\/j.mocell.2026.100406_bib102","doi-asserted-by":"crossref","first-page":"826","DOI":"10.1038\/s41419-025-08154-2","article-title":"Molecular hallmarks of neurodegeneration in polyglutamine spinocerebellar ataxias","volume":"16","author":"Nobrega","year":"2025","journal-title":"Cell Death Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib103","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1007\/s00401-019-02019-7","article-title":"Restoring brain cholesterol turnover improves autophagy and has therapeutic potential in mouse models of spinocerebellar ataxia","volume":"138","author":"Nobrega","year":"2019","journal-title":"Acta Neuropathol"},{"key":"10.1016\/j.mocell.2026.100406_bib104","doi-asserted-by":"crossref","first-page":"890","DOI":"10.1039\/D0NP00093K","article-title":"Oligodendroglial glycolipids in (Re)myelination: implications for multiple sclerosis research","volume":"38","author":"Nowack","year":"2021","journal-title":"Nat Prod Rep"},{"key":"10.1016\/j.mocell.2026.100406_bib105","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1016\/S0022-2275(20)39619-X","article-title":"Lipid composition of the normal human brain: gray matter, white matter, and myelin","volume":"6","author":"O'Brien","year":"1965","journal-title":"J Lipid Res"},{"key":"10.1016\/j.mocell.2026.100406_bib106","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/ng0793-221","article-title":"Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1","volume":"4","author":"Orr","year":"1993","journal-title":"Nat Genet"},{"key":"10.1016\/j.mocell.2026.100406_bib107","doi-asserted-by":"crossref","first-page":"1053","DOI":"10.1016\/S0092-8674(05)80010-8","article-title":"Acid sphingomyelinase-deficient mice mimic the neurovisceral form of human lysosomal storage disease (Niemann-Pick disease)","volume":"81","author":"Otterbach","year":"1995","journal-title":"Cell"},{"key":"10.1016\/j.mocell.2026.100406_bib108","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1186\/s40478-025-02054-4","article-title":"Astrocyte-neuron combined targeting for CYP46A1 gene therapy in Huntington's disease","volume":"13","author":"Parsai","year":"2025","journal-title":"Acta Neuropathol Commun"},{"key":"10.1016\/j.mocell.2026.100406_bib109","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1038\/nrn.2017.92","article-title":"Polyglutamine spinocerebellar ataxias - from genes to potential treatments","volume":"18","author":"Paulson","year":"2017","journal-title":"Nat Rev Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib110","doi-asserted-by":"crossref","first-page":"2179","DOI":"10.1016\/j.neurobiolaging.2014.03.020","article-title":"APOE epsilon2 allele may decrease the age at onset in patients with spinocerebellar ataxia type 3 or Machado-Joseph disease from the Chinese Han population","volume":"35","author":"Peng","year":"2014","journal-title":"Neurobiol Aging"},{"key":"10.1016\/j.mocell.2026.100406_bib111","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1016\/j.ymthe.2022.09.004","article-title":"Treatment with THI, an inhibitor of sphingosine-1-phosphate lyase, modulates glycosphingolipid metabolism and results therapeutically effective in experimental models of Huntington's disease","volume":"31","author":"Pepe","year":"2023","journal-title":"Mol Ther"},{"key":"10.1016\/j.mocell.2026.100406_bib112","doi-asserted-by":"crossref","DOI":"10.3390\/ijms24065956","article-title":"Treatment with the Glycosphingolipid Modulator THI Rescues Myelin Integrity in the Striatum of R6\/2 HD Mice","volume":"24","author":"Pepe","year":"2023","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.mocell.2026.100406_bib113","article-title":"Cholesteryl ester levels are elevated in the caudate and putamen of Huntington's disease patients","volume":"10","author":"Phillips","year":"2020","journal-title":"Sci Rep-Uk"},{"key":"10.1016\/j.mocell.2026.100406_bib114","doi-asserted-by":"crossref","DOI":"10.3390\/nu14102086","article-title":"Phospholipid Profiles Are Selectively Altered in the Putamen and White Frontal Cortex of Huntington's Disease","volume":"14","author":"Phillips","year":"2022","journal-title":"Nutrients"},{"key":"10.1016\/j.mocell.2026.100406_bib115","doi-asserted-by":"crossref","DOI":"10.1093\/braincomms\/fcab303","article-title":"The long and the short of Huntington's disease: how the sphingolipid profile is shifted in the caudate of advanced clinical cases","volume":"4","author":"Phillips","year":"2022","journal-title":"Brain Commun"},{"key":"10.1016\/j.mocell.2026.100406_bib116","doi-asserted-by":"crossref","DOI":"10.3390\/ijms241311001","article-title":"Cell-Type Specific Regulation of Cholesterogenesis by CYP46A1 Re-Expression in zQ175 HD Mouse Striatum","volume":"24","author":"Pinchaud","year":"2023","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.mocell.2026.100406_bib117","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1038\/s41467-017-00353-6","article-title":"Identifying therapeutic targets by combining transcriptional data with ordinal clinical measurements","volume":"8","author":"Pirhaji","year":"2017","journal-title":"Nat Commun"},{"key":"10.1016\/j.mocell.2026.100406_bib118","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1038\/nmeth.3940","article-title":"Revealing disease-associated pathways by network integration of untargeted metabolomics","volume":"13","author":"Pirhaji","year":"2016","journal-title":"Nat Methods"},{"key":"10.1016\/j.mocell.2026.100406_bib119","doi-asserted-by":"crossref","first-page":"1285","DOI":"10.1007\/BF00964847","article-title":"Levels of sulfatide synthesis distinguish oligodendroglia in different stages of maturation","volume":"10","author":"Poduslo","year":"1985","journal-title":"Neurochem Res"},{"key":"10.1016\/j.mocell.2026.100406_bib120","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1038\/ng1196-269","article-title":"Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2","volume":"14","author":"Pulst","year":"1996","journal-title":"Nat Genet"},{"key":"10.1016\/j.mocell.2026.100406_bib121","doi-asserted-by":"crossref","DOI":"10.1016\/j.nbd.2025.106827","article-title":"Cerebellar lipid dysregulation in SCA3: A comparative study in patients and mice","volume":"206","author":"Putka","year":"2025","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib122","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1136\/jnnp-2015-311305","article-title":"Non-neural phenotype of spinal and bulbar muscular atrophy: results from a large cohort of Italian patients","volume":"87","author":"Querin","year":"2016","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"10.1016\/j.mocell.2026.100406_bib123","doi-asserted-by":"crossref","DOI":"10.3390\/cells10030565","article-title":"Oligodendrocyte Dysfunction in Amyotrophic Lateral Sclerosis: Mechanisms and Therapeutic Perspectives","volume":"10","author":"Raffaele","year":"2021","journal-title":"Cells"},{"key":"10.1016\/j.mocell.2026.100406_bib124","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1016\/j.devcel.2015.01.029","article-title":"Fatty acid trafficking in starved cells: regulation by lipid droplet lipolysis, autophagy, and mitochondrial fusion dynamics","volume":"32","author":"Rambold","year":"2015","journal-title":"Dev Cell"},{"key":"10.1016\/j.mocell.2026.100406_bib125","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1093\/hmg\/ddn310","article-title":"Mitochondrial abnormalities in spinal and bulbar muscular atrophy","volume":"18","author":"Ranganathan","year":"2009","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.mocell.2026.100406_bib126","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1007\/s00401-016-1550-4","article-title":"Glycolytic-to-oxidative fiber-type switch and mTOR signaling activation are early-onset features of SBMA muscle modified by high-fat diet","volume":"132","author":"Rocchi","year":"2016","journal-title":"Acta Neuropathol"},{"key":"10.1016\/j.mocell.2026.100406_bib127","doi-asserted-by":"crossref","first-page":"1747","DOI":"10.1080\/1028415X.2021.1895479","article-title":"Weight loss is correlated with disease severity in Spinocerebellar ataxia type 2: a cross-sectional cohort study","volume":"25","author":"Rodriguez-Grana","year":"2022","journal-title":"Nutr Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib128","doi-asserted-by":"crossref","first-page":"1026","DOI":"10.1007\/s00415-018-8790-2","article-title":"The metabolic and endocrine characteristics in spinal and bulbar muscular atrophy","volume":"265","author":"Rosenbohm","year":"2018","journal-title":"J Neurol"},{"key":"10.1016\/j.mocell.2026.100406_bib129","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1016\/S0896-6273(02)00872-3","article-title":"Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders","volume":"35","author":"Ross","year":"2002","journal-title":"Neuron"},{"key":"10.1016\/j.mocell.2026.100406_bib130","doi-asserted-by":"crossref","first-page":"734","DOI":"10.1038\/s41598-018-19160-0","article-title":"Alteration in Fluidity of Cell Plasma Membrane in Huntington Disease Revealed by Spectral Phasor Analysis","volume":"8","author":"Sameni","year":"2018","journal-title":"Sci Rep"},{"key":"10.1016\/j.mocell.2026.100406_bib131","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1093\/jnen\/60.2.161","article-title":"Early and progressive accumulation of reactive microglia in the Huntington disease brain","volume":"60","author":"Sapp","year":"2001","journal-title":"J Neuropathol Exp Neurol"},{"key":"10.1016\/j.mocell.2026.100406_bib132","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1016\/0163-7827(85)90011-6","article-title":"Lipids of nervous tissue: composition and metabolism","volume":"24","author":"Sastry","year":"1985","journal-title":"Prog Lipid Res"},{"key":"10.1016\/j.mocell.2026.100406_bib133","doi-asserted-by":"crossref","DOI":"10.1523\/ENEURO.0421-18.2019","article-title":"Sphingomyelin in Brain and Cognitive Development: Preliminary Data","volume":"6","author":"Schneider","year":"2019","journal-title":"eNeuro"},{"key":"10.1016\/j.mocell.2026.100406_bib134","doi-asserted-by":"crossref","first-page":"1493","DOI":"10.1038\/jcbfm.2013.128","article-title":"Why does brain metabolism not favor burning of fatty acids to provide energy? Reflections on disadvantages of the use of free fatty acids as fuel for brain","volume":"33","author":"Schonfeld","year":"2013","journal-title":"J Cereb Blood Flow Metab"},{"key":"10.1016\/j.mocell.2026.100406_bib135","doi-asserted-by":"crossref","DOI":"10.1523\/JNEUROSCI.1954-20.2021","article-title":"Impaired oligodendrocyte maturation is an early feature in SCA3 disease pathogenesis","volume":"42","author":"Schuster","year":"2022","journal-title":"Journal of Neuroscience"},{"key":"10.1016\/j.mocell.2026.100406_bib136","doi-asserted-by":"crossref","DOI":"10.3390\/ijms20235854","article-title":"Human and Mouse Spino-Cerebellar Tissue, Ataxin-2 Expansion Affects Ceramide-Sphingomyelin Metabolism","volume":"20","author":"Sen","year":"2019","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.mocell.2026.100406_bib137","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.yclnex.2018.05.001","article-title":"Lipids: An insight into the neurodegenerative disorders","volume":"20","author":"Shamim","year":"2018","journal-title":"Clinical Nutrition Experimental"},{"key":"10.1016\/j.mocell.2026.100406_bib138","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.nbd.2016.11.013","article-title":"Early and brain region-specific decrease of de novo cholesterol biosynthesis in Huntington's disease: A cross-validation study in Q175 knock-in mice","volume":"98","author":"Shankaran","year":"2017","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib139","doi-asserted-by":"crossref","DOI":"10.1016\/j.nbd.2023.106313","article-title":"Early whole-body mutant huntingtin lowering averts changes in proteins and lipids important for synapse function and white matter maintenance in the LacQ140 mouse model","volume":"187","author":"Shing","year":"2023","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib140","doi-asserted-by":"crossref","DOI":"10.1016\/j.bbadis.2021.166127","article-title":"Deciphering the key mechanisms leading to alteration of lipid metabolism in Drosophila model of Huntington's disease","volume":"1867","author":"Singh","year":"2021","journal-title":"Biochim Biophys Acta Mol Basis Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib141","doi-asserted-by":"crossref","DOI":"10.3389\/fnins.2020.572965","article-title":"Gangliosides in the Brain: Physiology, Pathophysiology and Therapeutic Applications","volume":"14","author":"Sipione","year":"2020","journal-title":"Front Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib142","doi-asserted-by":"crossref","first-page":"1369","DOI":"10.4103\/1673-5374.165498","article-title":"The choline pathway as a strategy to promote central nervous system (CNS) remyelination","volume":"10","author":"Skripuletz","year":"2015","journal-title":"Neural Regen Res"},{"key":"10.1016\/j.mocell.2026.100406_bib143","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1007\/s12035-026-05726-x","article-title":"Single-Cell RNA Sequencing Reveals Impaired CHIP-Mediated Heat Stress Response in SCA3 Pathogenesis","volume":"63","author":"Tang","year":"2026","journal-title":"Mol Neurobiol"},{"key":"10.1016\/j.mocell.2026.100406_bib144","doi-asserted-by":"crossref","first-page":"2","DOI":"10.3389\/fncel.2019.00002","article-title":"Phosphatidic Acid: From Pleiotropic Functions to Neuronal Pathology","volume":"13","author":"Tanguy","year":"2019","journal-title":"Front Cell Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib145","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1016\/j.neuron.2023.10.039","article-title":"Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1","volume":"112","author":"Tejwani","year":"2024","journal-title":"Neuron"},{"key":"10.1016\/j.mocell.2026.100406_bib146","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.mocell.2026.100406_bib147","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1186\/s13024-018-0261-9","article-title":"Transcriptional profiling and biomarker identification reveal tissue specific effects of expanded ataxin-3 in a spinocerebellar ataxia type 3 mouse model","volume":"13","author":"Toonen","year":"2018","journal-title":"Mol Neurodegener"},{"key":"10.1016\/j.mocell.2026.100406_bib148","doi-asserted-by":"crossref","first-page":"10","DOI":"10.3389\/fnmol.2018.00010","article-title":"Neuronal Lipid Metabolism: Multiple Pathways Driving Functional Outcomes in Health and Disease","volume":"11","author":"Tracey","year":"2018","journal-title":"Front Mol Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib149","doi-asserted-by":"crossref","first-page":"3578","DOI":"10.1093\/hmg\/ddl434","article-title":"Mutant huntingtin inhibits clathrin-independent endocytosis and causes accumulation of cholesterol in vitro and in vivo","volume":"15","author":"Trushina","year":"2006","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.mocell.2026.100406_bib150","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/j.jbior.2014.09.010","article-title":"Diacylglycerol, phosphatidic acid, and their metabolic enzymes in synaptic vesicle recycling","volume":"57","author":"Tu-Sekine","year":"2015","journal-title":"Adv Biol Regul"},{"key":"10.1016\/j.mocell.2026.100406_bib151","doi-asserted-by":"crossref","first-page":"2187","DOI":"10.1093\/hmg\/ddm170","article-title":"Cholesterol biosynthesis pathway is disturbed in YAC128 mice and is modulated by huntingtin mutation","volume":"16","author":"Valenza","year":"2007","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.mocell.2026.100406_bib152","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.nbd.2007.07.004","article-title":"Progressive dysfunction of the cholesterol biosynthesis pathway in the R6\/2 mouse model of Huntington's disease","volume":"28","author":"Valenza","year":"2007","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib153","doi-asserted-by":"crossref","first-page":"690","DOI":"10.1038\/cdd.2014.162","article-title":"Disruption of astrocyte-neuron cholesterol cross talk affects neuronal function in Huntington's disease","volume":"22","author":"Valenza","year":"2015","journal-title":"Cell Death Differ"},{"key":"10.1016\/j.mocell.2026.100406_bib154","doi-asserted-by":"crossref","first-page":"9932","DOI":"10.1523\/JNEUROSCI.3355-05.2005","article-title":"Dysfunction of the cholesterol biosynthetic pathway in Huntington's disease","volume":"25","author":"Valenza","year":"2005","journal-title":"J Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib155","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1016\/j.bbalip.2010.02.007","article-title":"Formation and function of apolipoprotein E-containing lipoproteins in the nervous system","volume":"1801","author":"Vance","year":"2010","journal-title":"Biochim Biophys Acta"},{"key":"10.1016\/j.mocell.2026.100406_bib156","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1093\/cvr\/cvu148","article-title":"HDL and cholesterol handling in the brain","volume":"103","author":"Vitali","year":"2014","journal-title":"Cardiovasc Res"},{"key":"10.1016\/j.mocell.2026.100406_bib157","doi-asserted-by":"crossref","first-page":"187","DOI":"10.3233\/JHD-150149","article-title":"Mass Spectrometry Analysis of Wild-Type and Knock-in Q140\/Q140 Huntington's Disease Mouse Brains Reveals Changes in Glycerophospholipids Including Alterations in Phosphatidic Acid and Lyso-Phosphatidic Acid","volume":"4","author":"Vodicka","year":"2015","journal-title":"J Huntingtons Dis"},{"key":"10.1016\/j.mocell.2026.100406_bib158","doi-asserted-by":"crossref","first-page":"3443","DOI":"10.1038\/s41467-021-23840-3","article-title":"Saturated free fatty acids and association with memory formation","volume":"12","author":"Wallis","year":"2021","journal-title":"Nat Commun"},{"key":"10.1016\/j.mocell.2026.100406_bib159","doi-asserted-by":"crossref","DOI":"10.3389\/fphys.2014.00231","article-title":"Metabolic and hormonal signatures in pre-manifest and manifest Huntington's disease patients","volume":"5","author":"Wang","year":"2014","journal-title":"Front Physiol"},{"key":"10.1016\/j.mocell.2026.100406_bib160","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1016\/j.devcel.2013.01.013","article-title":"Triacylglycerol synthesis enzymes mediate lipid droplet growth by relocalizing from the ER to lipid droplets","volume":"24","author":"Wilfling","year":"2013","journal-title":"Dev Cell"},{"key":"10.1016\/j.mocell.2026.100406_bib161","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1186\/s41232-023-00289-z","article-title":"Lipid in microglial biology - from material to mediator","volume":"43","author":"Yamamoto","year":"2023","journal-title":"Inflamm Regen"},{"key":"10.1016\/j.mocell.2026.100406_bib162","doi-asserted-by":"crossref","first-page":"159","DOI":"10.3389\/fnmol.2019.00159","article-title":"Metabolic Profiling Reveals Biochemical Pathways and Potential Biomarkers of Spinocerebellar Ataxia 3","volume":"12","author":"Yang","year":"2019","journal-title":"Front Mol Neurosci"},{"key":"10.1016\/j.mocell.2026.100406_bib163","doi-asserted-by":"crossref","first-page":"1230","DOI":"10.1007\/s11064-012-0744-y","article-title":"Functional roles of gangliosides in neurodevelopment: an overview of recent advances","volume":"37","author":"Yu","year":"2012","journal-title":"Neurochem Res"},{"key":"10.1016\/j.mocell.2026.100406_bib164","doi-asserted-by":"crossref","first-page":"1192","DOI":"10.1194\/jlr.R046458","article-title":"LPA receptor signaling: pharmacology, physiology, and pathophysiology","volume":"55","author":"Yung","year":"2014","journal-title":"J Lipid Res"},{"key":"10.1016\/j.mocell.2026.100406_bib165","doi-asserted-by":"crossref","first-page":"43553","DOI":"10.1074\/jbc.C200551200","article-title":"Gluing the respiratory chain together. Cardiolipin is required for supercomplex formation in the inner mitochondrial membrane","volume":"277","author":"Zhang","year":"2002","journal-title":"J Biol Chem"},{"key":"10.1016\/j.mocell.2026.100406_bib166","doi-asserted-by":"crossref","DOI":"10.1002\/smsc.202500152","article-title":"Astrocyte Lipid Droplet Dynamics Orchestrate Neurological Disorders and Therapeutic Horizons","volume":"5","author":"Zhong","year":"2025","journal-title":"Small Sci"}],"container-title":["Molecules and Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S101684782600097X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S101684782600097X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T19:37:21Z","timestamp":1792093041000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S101684782600097X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10]]},"references-count":166,"alternative-id":["S101684782600097X"],"URL":"https:\/\/doi.org\/10.1016\/j.mocell.2026.100406","relation":{},"ISSN":["1016-8478"],"issn-type":[{"value":"1016-8478","type":"print"}],"subject":[],"published":{"date-parts":[[2026,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Lipid dysregulation in polyglutamine diseases","name":"articletitle","label":"Article Title"},{"value":"Molecules and Cells","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.mocell.2026.100406","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 The Author(s). Published by Elsevier Inc. on behalf of Korean Society for Molecular and Cellular Biology.","name":"copyright","label":"Copyright"}],"article-number":"100406"}}