Ontology highlight
ABSTRACT:
SUBMITTER: Fernandez-Vizarra E
PROVIDER: S-EPMC5969560 | biostudies-literature | 2018
REPOSITORIES: biostudies-literature
Fernandez-Vizarra Erika E Zeviani Massimo M
Cell cycle (Georgetown, Tex.) 20180410 6
Regulation of the mitochondrial respiratory chain biogenesis is a matter of great interest because of its implications for mitochondrial disease. One of the mitochondrial disease genes recently discovered associated to encephalopathy and mitochondrial complex III (cIII) deficiency is TTC19. Our study of TTC19-deficient human and mouse models, has led us to propose a post-assembly quality control role or 'husbandry' function for this factor that is linked to Rieske Fe-S protein (UQCRFS1). UQCRFS1 ...[more]