In a previous work, using cellular models derived from PKAN patients, we confirmed the hypothesis that CoA deficiency caused by PANK2 mutations affects the expression levels and activity of key mitochondrial proteins harboring a 4′-phosphopantetheinyl cofactor such as mtACP, ALDH1L2 or AASS [12]. This evidence concerns the gene AASS and pantothenate kinase-associated neurodegeneration.