Recently, it has been demonstrated that CoA-dependent activation of mitochondrial acyl carrier protein (mtACP) is a possible process linking several neurodegenerative diseases such as PKAN, CoPAN (CoA synthase protein-associated neurodegeneration), MePAN (Mitochondrial enoyl CoA reductase protein-associated neurodegeneration), and PDH-E2 (pyruvate dehydrogenase-E2) deficiency which share key phenotypic features but harbor defects in distinct metabolic processes [12]. Here, NDUFAB1 is linked to neurodegenerative disease.