COASY and neurodegeneration with brain iron accumulation: Mutations in PANK2 cause PKAN (Pantothenate Kinase-Associated Neurodegeneration, MIM 234200), which is the most frequent NBIA form (35–50% of NBIA patients), and deficiency of COASY leads to COPAN (COasy Protein-Associated Neurodegeneration, MIM 615643), which is an ultra-rare form of NBIA (Table 1).