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We demonstrate that TDP-43, the pathological signature protein for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), influences cholesterol metabolism in oligodendrocytes. TDP-43 binds directly to mRNA of SREBF2, the master transcription regulator for cholesterol metabolism, and multiple mRNAs encoding proteins responsible for cholesterol biosynthesis and uptake, including HMGCR, HMGCS1, and LDLR. TDP-43 depletion leads to reduced SREBF2 and LDLR expression, and cholesterol levels in vitro and in vivo. TDP-43\u2013mediated changes in cholesterol levels can be restored by reintroducing SREBF2 or LDLR. Additionally, cholesterol supplementation rescues demyelination caused by TDP-43 deletion. Furthermore, oligodendrocytes harboring TDP-43 pathology from FTD patients show reduced HMGCR and HMGCS1, and coaggregation of LDLR and TDP-43. Collectively, our results indicate that TDP-43 plays a role in cholesterol homeostasis in oligodendrocytes, and cholesterol dysmetabolism may be implicated in TDP-43 proteinopathies\u2013related diseases.<\/jats:p>","DOI":"10.1083\/jcb.201910213","type":"journal-article","created":{"date-parts":[[2021,5,29]],"date-time":"2021-05-29T02:28:26Z","timestamp":1622255306000},"update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":64,"title":["TDP-43 mediates SREBF2-regulated gene expression required for oligodendrocyte myelination"],"prefix":"10.1083","volume":"220","author":[{"given":"Wan Yun","family":"Ho","sequence":"first","affiliation":[{"name":"Department of Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jer-Cherng","family":"Chang","sequence":"additional","affiliation":[{"name":"Department of Physiology, Yong Loo Lin School 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