Isovaleroylglycine is a compound found in the urine of patients with isovaleric acidemia, a disorder created by defective leucine metabolism due to a deficiency in the isovaleryl coenzyme A dehydrogenase.{28777} It has been used as a diagnostic tool in the diagnosis of various acidemias and mitochondrial fatty acid β-oxidation defects.
产品描述
Urinary metabolite of leucine that is used as a diagnostic tool in the diagnosis of various acidemias and mitochondrial fatty acid β-oxidation defects