The progeroid gene BubR1 regulates axon myelination and motor function.

ABSTRACT: Myelination, the process by which oligodendrocytes form the myelin sheath around axons, is key to axonal signal transduction and related motor function in the central nervous system (CNS). Aging is characterized by degenerative changes in the myelin sheath, although the molecular underpinnings of normal and aberrant myelination remain incompletely understood. Here we report that axon myelination and related motor function are dependent on BubR1, a mitotic checkpoint protein that has been linked to progeroid phenotypes when expressed at low levels and healthy lifespan when overabundant. We found that oligodendrocyte progenitor cell proliferation and oligodendrocyte density is markedly reduced in mutant mice with low amounts of BubR1 (BubR1^H/H mice), causing axonal hypomyel

SUBMITTER: Choi CI 

PROVIDER: S-EPMC5191862 | biostudies-literature | 2016 Sep

REPOSITORIES: biostudies-literature