Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease.

ABSTRACT:

Background

Huntington Disease (HD) is a neurodegenerative disorder in which caspase activation and cleavage of substrates, including the huntingtin protein, has been invoked as a pathological mechanism. Specific changes in caspase-2 (casp2) activity have been suggested to contribute to the pathogenesis of HD, however unique casp2 cleavage substrates have remained elusive. We thus utilized mice completely lacking casp2 (casp2-/-) to examine the role played by casp2 in the progression of HD. This 'substrate agnostic' approach allows us to query the effect of casp2 on HD progression without pre-defining proteolytic substrates of interest.

Results

YAC128 HD model mice lacking casp2 show protection from well-validated motor and cognitive features of HD, including performance on r

SUBMITTER: Carroll JB 

PROVIDER: S-EPMC3180273 | biostudies-literature | 2011 Aug

REPOSITORIES: biostudies-literature