Glutaric aciduria type 1 (GA1, OMIM#231670) is a rare  neurometabolic disorder of lysine, hydroxylysine and tryptophan metabolism caused by profound deficiency of the mitochondrial enzyme, glutaryl‐CoA dehydrogenase (GCDH; EC number: 1.3.99.7). The gene discussed is GCDH; the disease is glutaryl-CoA dehydrogenase deficiency.