In our previous works, using cellular models derived from PKAN patients, we confirmed the hypothesis that CoA deficiency caused by PANK2 variants affects the expression levels and activity of key mitochondrial proteins harboring a 4′-phosphopantetheiny cofactor such as mtACP (mitochondrial acyl carrier protein), ALDH1L2 (mitochondrial 10-Formyltetrahydrofolate Dehydrogenase) or AASS (alpha-aminoadipic semialdehyde synthase) [14]. The gene discussed is AASS; the disease is pantothenate kinase-associated neurodegeneration.