Glutaric aciduria type 1 (GA1; OMIM #231670) is a rare neurometabolic disorder of L-lysine metabolism caused by inherited deficiency of glutaryl-CoA dehydrogenase (GCDH) resulting in accumulation of glutaric acid (GA), 3-hydroxyglutaric acid and glutarylcarnitine especially within the brain. Here, GCDH is linked to glutaryl-CoA dehydrogenase deficiency.