While a connection between a lipid transporting protein and brain iron may be unexpected, mutations in a range of lipid metabolism genes are a surprisingly common cause of Neurodegeneration with Brain Iron Accumulation (NBIA) including mutations in pantothenate kinase 2 (PANK2), phospholipase A2 group VI (PLA2G6), fatty acid 2-hydroxylase (FA2H) and CoA synthase (COASY) [8, 9]. Here, COASY is linked to Neurodegeneration.