Mediator MED23 controls oligodendrogenesis and myelination by modulating Sp1/P300-directed gene programs.

ABSTRACT: Gaining the molecular understanding for myelination development and regeneration has been a long-standing goal in neurological research. Mutations in the transcription cofactor Mediator Med23 subunit are often associated with intellectual disability and white matter defects, although the precise functions and mechanisms of Mediator in myelination remain unclear. In this study, we generated a mouse model carrying an Med23^Q649R mutation that has been identified in a patient with hypomyelination features. The MED23^Q649R mouse model develops white matter thinning and cognitive decline, mimicking common clinical phenotypes. Further, oligodendrocyte-lineage specific Med23 knockout mice verified the important function of MED23 in regulating central nervous system myelination

SUBMITTER: Zhang S 

PROVIDER: S-EPMC11473658 | biostudies-literature | 2024 Oct

REPOSITORIES: biostudies-literature