Adenosine A_2A receptor stimulation restores cell functions and differentiation in Niemann-Pick type C-like oligodendrocytes.

ABSTRACT: Niemann Pick type C (NPC) disease is a rare neurovisceral disorder. Mutations in npc1 gene induce an intracellular accumulation of unesterified cholesterol in the endosomal/lysosomal system causing cell death. We recently showed that stimulation of adenosine A_2A receptors (A_2AR) restores cholesterol accumulation in late endosomes/lysosomes in human NPC fibroblasts and neural cell lines transiently transfected with NPC1 siRNA, suggesting that these receptors might be targeted to contrast the disease. Since NPC1 disease is characterized by dysmyelination and maturational arrest of oligodendrocyte progenitors (OPs), in this study, we investigated whether A_2AR stimulation could promote oligodendrocyte differentiation and myelin formation, thus overcoming these

SUBMITTER: De Nuccio C 

PROVIDER: S-EPMC6611770 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature