Disruption of polyubiquitin gene Ubb causes dysregulation of neural stem cell differentiation with premature gliogenesis.

ABSTRACT: Disruption of polyubiquitin gene Ubb leads to early-onset reactive gliosis and adult-onset hypothalamic neurodegeneration in mice. However, it remains unknown why reduced levels of ubiquitin (Ub) due to loss of Ubb lead to these neural phenotypes. To determine whether or not the defects in neurons or their progenitors per se, but not in their cellular microenvironment, are the cause of the neural phenotypes observed in Ubb(-/-) mice, we investigated the properties of cultured cells isolated from Ubb(-/-) mouse embryonic brains. Although cells were cultured under conditions promoting neuronal growth, Ubb(-/-) cells underwent apoptosis during culture in vitro, with increased numbers of glial cells and decreased numbers of neurons. Intriguingly, at the beginning of the Ubb(-/-) cell culture,

SUBMITTER: Ryu HW 

PROVIDER: S-EPMC4229674 | biostudies-literature | 2014 Nov

REPOSITORIES: biostudies-literature