Reduced Levels of Proteasome Products in a Mouse Striatal Cell Model of Huntington's Disease.

ABSTRACT: Huntington's disease is the result of a long polyglutamine tract in the gene encoding huntingtin protein, which in turn causes a large number of cellular changes and ultimately results in neurodegeneration of striatal neurons. Although many theories have been proposed, the precise mechanism by which the polyglutamine expansion causes cellular changes is not certain. Some evidence supports the hypothesis that the long polyglutamine tract inhibits the proteasome, a multiprotein complex involved in protein degradation. However, other studies report normal proteasome function in cells expressing long polyglutamine tracts. The controversy may be due to the methods used to examine proteasome activity in each of the previous studies. In the present study, we measured proteasome function by examin

SUBMITTER: Dasgupta S 

PROVIDER: S-EPMC4686214 | biostudies-literature | 2015

REPOSITORIES: biostudies-literature