[HTML][HTML] Rhes, a striatal-selective protein implicated in Huntington disease, binds beclin-1 and activates autophagy
RG Mealer, AJ Murray, N Shahani… - Journal of Biological …, 2014 - ASBMB
The protein mutated in Huntington disease (HD), mutant huntingtin (mHtt), is expressed
throughout the brain and body. However, the pathology of HD is characterized by early and
dramatic destruction selectively of the striatum. We previously reported that the striatal-
specific protein Rhes binds mHtt and enhances its cytotoxicity. Moreover, Rhes-deleted mice
are dramatically protected from neurodegeneration and motor dysfunction in mouse models
of HD. We now report a function of Rhes in autophagy, a lysosomal degradation pathway …
throughout the brain and body. However, the pathology of HD is characterized by early and
dramatic destruction selectively of the striatum. We previously reported that the striatal-
specific protein Rhes binds mHtt and enhances its cytotoxicity. Moreover, Rhes-deleted mice
are dramatically protected from neurodegeneration and motor dysfunction in mouse models
of HD. We now report a function of Rhes in autophagy, a lysosomal degradation pathway …