[HTML][HTML] LRP130 protein remodels mitochondria and stimulates fatty acid oxidation
L Liu, M Sanosaka, S Lei, ML Bestwick, JH Frey… - Journal of Biological …, 2011 - ASBMB
Impaired oxidative phosphorylation (OXPHOS) is implicated in several metabolic disorders.
Even though mitochondrial DNA encodes several subunits critical for OXPHOS, the
metabolic consequence of activating mitochondrial transcription remains unclear. We show
here that LRP130, a protein involved in Leigh syndrome, increases hepatic β-fatty acid
oxidation. Using convergent genetic and biochemical approaches, we demonstrate LRP130
complexes with the mitochondrial RNA polymerase to activate mitochondrial transcription …
Even though mitochondrial DNA encodes several subunits critical for OXPHOS, the
metabolic consequence of activating mitochondrial transcription remains unclear. We show
here that LRP130, a protein involved in Leigh syndrome, increases hepatic β-fatty acid
oxidation. Using convergent genetic and biochemical approaches, we demonstrate LRP130
complexes with the mitochondrial RNA polymerase to activate mitochondrial transcription …