E3 ubiquitin-protein ligase NHLRC1 (Nhlrc1)

E3 ubiquitin-protein ligase. Together with the phosphatase EPM2A/laforin, seems to be involved in the clearance of toxic polyglucosan and protein aggregates via multiple pathways.

In complex with EPM2A/laforin and HSP70, suppresses the cellular toxicity of misfolded proteins by promoting their degradation during the ubiquitin-proteasome system (UPS). Ubiquitinates the glycogen-targeting protein phosphatase subunits PPP1R3C/PTG and PPP1R3D at a laforin-dependent manner and targets for proteasome-dependent degradation, thus decreasing glycogen accumulation.