{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"omics_type":["Unknown"],"volume":["2(3-4)"],"submitter":["Lin ST"],"pubmed_abstract":["Duplication of the gene encoding lamin B1 (LMNB1) with increased mRNA and protein levels has been shown to cause severe myelin loss in the brains of adult-onset autosomal dominant leukodystrophy patients. Similar to many neurodegenerative disorders, patients with adult-onset autosomal dominant leukodystrophy are phenotypically normal until adulthood and the defect is specific to the central nervous system despite the ubiquitous expression pattern of lamin B1. We set out to dissect the molecular mechanisms underlying this demyelinating phenotype. Increased lamin B1 expression results in disturbances of inner nuclear membrane proteins, chromatin organization and nuclear pore transport in vitro. It also leads to premature arrest of oligodendrocyte differentiation, which might be caused by red"],"journal":["Disease models & mechanisms"],"pagination":["178-88"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC2650193"],"repository":["biostudies-literature"],"pubmed_title":["miR-23 regulation of lamin B1 is crucial for oligodendrocyte development and myelination."],"pmcid":["PMC2650193"],"pubmed_authors":["Fu YH","Lin ST"],"additional_accession":[]},"is_claimable":false,"name":"miR-23 regulation of lamin B1 is crucial for oligodendrocyte development and myelination.","description":"Duplication of the gene encoding lamin B1 (LMNB1) with increased mRNA and protein levels has been shown to cause severe myelin loss in the brains of adult-onset autosomal dominant leukodystrophy patients. Similar to many neurodegenerative disorders, patients with adult-onset autosomal dominant leukodystrophy are phenotypically normal until adulthood and the defect is specific to the central nervous system despite the ubiquitous expression pattern of lamin B1. We set out to dissect the molecular mechanisms underlying this demyelinating phenotype. Increased lamin B1 expression results in disturbances of inner nuclear membrane proteins, chromatin organization and nuclear pore transport in vitro. It also leads to premature arrest of oligodendrocyte differentiation, which might be caused by red","dates":{"release":"2009-01-01T00:00:00Z","publication":"2009 Mar-Apr","modification":"2025-04-04T13:40:01.215Z","creation":"2019-03-27T00:20:59Z"},"accession":"S-EPMC2650193","cross_references":{"pubmed":["19259393"],"doi":["10.1242/dmm.001065"]}}