The Huntington's disease-related cardiomyopathy prevents a hypertrophic response in the R6/2 mouse model.

ABSTRACT: Huntington's disease (HD) is neurodegenerative disorder for which the mutation results in an extra-long tract of glutamines that causes the huntingtin protein to aggregate. It is characterized by neurological symptoms and brain pathology that is associated with nuclear and cytoplasmic aggregates and with transcriptional deregulation. Despite the fact that HD has been recognized principally as a neurological disease, there are multiple epidemiological studies showing that HD patients exhibit a high rate of cardiovascular events leading to heart failure. To unravel the mechanistic basis of cardiac dysfunction in HD, we employed a wide range of molecular techniques using the well-established genetic R6/2 mouse model that develop a considerable degree of the cardiac atrophy at end stage diseas

SUBMITTER: Mielcarek M 

PROVIDER: S-EPMC4182603 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature