Glycogen synthase protects neurons from cytotoxicity of mutant huntingtin by enhancing the autophagy flux.

ABSTRACT: Healthy neurons do not store glycogen while they do possess the machinery for the glycogen synthesis albeit at an inactive state. Neurons in the degenerating brain, however, are known to accumulate glycogen, although its significance was not well understood. Emerging reports present contrasting views on neuronal glycogen synthesis; a few reports demonstrate a neurotoxic effect of glycogen while a few others suggest glycogen to be neuroprotective. Thus, the specific role of glycogen and glycogen synthase in neuronal physiology is largely unexplored. Using cellular and animal models of Huntington's disease, we show here that the overexpression of cytotoxic mutant huntingtin protein induces glycogen synthesis in the neurons by activating glycogen synthase and the overexpressed glycogen syntha

SUBMITTER: Rai A 

PROVIDER: S-EPMC5833817 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature