The dietary management consists of a diet low in Lysine and Tryptophan (i.e. in native protein). Such low protein diet requires the addition of protein supplements free from lysine and low in Tryptophan, additionally enriched with carnitine.
Glutaric Aciduria Type I
Glutaric Aciduria Type I is a rare inherited metabolic disorder in protein metabolism, caused by a defect of the enzyme Glutaryl-CoA dehydrogenase, which is responsible for the degradation of the amino acids Lysine, Hydroxylysine and Tryptophan. The consequence is an accumulation of the metabolites Glutaric acid, 3-hydroxyglutaric acid, glutaconic acid and glutarylcarnitine in the body, associated with a decrease of carnitine due to increased urinary excretion.