GCDH and glutaryl-CoA dehydrogenase deficiency: Glutaric aciduria type 1 (GA1) is an autosomal recessive disorder caused by deficiency of the enzyme, glutaryl-CoA dehydrogenase (GCDH), which leads to the accumulation of glutarate, glutarylcarnitine, 3-hydroxyglutarate, and glutaconate in various tissues, blood, CSF, urine, and multiple organs [70].