Novel proteomic changes in brain mitochondria provide insights into mitochondrial dysfunction in mouse models of Huntington's disease.

ABSTRACT: Huntington's disease (HD) is a progressive ultimately fatal disorder caused by a glutamine-encoding CAG expansion in the huntingtin (HTT) gene that results in degeneration mainly in striatal and cerebro-cortical brain regions. Mitochondrial dysfunction is one important facet of HD pathogenesis. Here we used R6/2 and YAC128 HD mouse models of human HD, that express different HTT transgenes and have different progression rates, to identify HD brain mitochondrial proteomic signatures. Cerebral cortical mitochondrial preparations from HD and wild-type litter mate mice were compared by two-dimensional SDS-PAGE electrophoresis and MALDI-TOF/TOF mass spectrometry. Proteomic analyses inferred 17 and 12 differentially expressed proteins, respectively in 12 week R6/2 and 15 month YAC128 HD mice, com

SUBMITTER: Agrawal S 

PROVIDER: S-EPMC7011782 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature