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]. The gene discussed is ALDH1L2; the disease is pantothenate kinase-associated neurodegeneration.