Making the most of what you've got: optimizing residual OXPHOS function in mitochondrial diseases.
Patients affected by mitochondrial OXPHOS disorders are still faced with a grim lack of therapeutic options. In this Closeup, Carlos Moraes revisits the recent data by Giovanni Manfredi on PKA's functions in the mitochondria and now its modulation can improve respiration and ATP production in COX-defective cells.
Moraes
Carlos T
CT
Department of Neurology, University of Miami Miller School of Medicine, Miami, FL,USA. cmoraes@med.miami.edu
D000255:Adenosine Triphosphate / Q000378:metabolism; D017868:Cyclic AMP-Dependent Protein Kinases / Q000378:metabolism; D003576:Electron Transport Complex IV / Q000378:metabolism; D006801:Humans; D008928:Mitochondria / Q000201:enzymology* / Q000473:pathology*; D028361:Mitochondrial Diseases / Q000201:enzymology*; D010085:Oxidative Phosphorylation
D000255:Adenosine Triphosphate; D003576:Electron Transport Complex IV; D017868:Cyclic AMP-Dependent Protein Kinases