Ontology highlight
ABSTRACT:
SUBMITTER: Chaturvedi RK
PROVIDER: S-EPMC2733807 | biostudies-other | 2009 Aug
REPOSITORIES: biostudies-other
Chaturvedi Rajnish K RK Adhihetty Peter P Shukla Shubha S Hennessy Thomas T Calingasan Noel N Yang Lichuan L Starkov Anatoly A Kiaei Mahmoud M Cannella Milena M Sassone Jenny J Ciammola Andrea A Squitieri Fernando F Beal M Flint MF
Human molecular genetics 20090521 16
We investigated the role of PPAR gamma coactivator 1alpha (PGC-1alpha) in muscle dysfunction in Huntington's disease (HD). We observed reduced PGC-1alpha and target genes expression in muscle of HD transgenic mice. We produced chronic energy deprivation in HD mice by administering the catabolic stressor beta-guanidinopropionic acid (GPA), a creatine analogue that reduces ATP levels, activates AMP-activated protein kinase (AMPK), which in turn activates PGC-1alpha. Treatment with GPA resulted in ...[more]