Article
Differential expression of PGC-1α and metabolic sensors suggest age-dependent induction of mitochondrial biogenesis in Friedreich ataxia fibroblasts.
PloS one - 1 Jan 2011
García-Giménez José Luis, Gimeno Amparo, Gonzalez-Cabo Pilar, Dasí Francisco, Bolinches-Amorós Arantxa, Mollá Belén, Palau Francesc, Pallardó Federico V
Abstract excerpt
BACKGROUND: Friedreich's ataxia (FRDA) is a mitochondrial rare disease, which molecular origin is associated with defect in the expression of frataxin. The pathological consequences are degeneration of nervous system structures and cardiomyopathy with necrosis and fibrosis, among others. PRINCIPAL FINDINGS: Using FRDA fibroblasts we have characterized the oxidative stress status and mitochondrial biogenesis. We...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.