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NFPA 704
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2
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N-ISOVALERYLGLYCINE C화학적 특성, 용도, 생산
개요
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. It has been used as a diagnostic tool in the diagnosis of various acidemias and mitochondrial fatty acid β-oxidation defects.
화학적 성질
White to Off-White Solid
용도
N-Isovalerylglycine is a useful diagnostic tool in the diagnosis of many organic acidemias and mitochondrial fatty acid β-oxidation defects. N-Isovalerylglycine is found in the urine of patients with
isovaleric acidemia, an inborn error of leucine metabolism.
정의
ChEBI: An N-acylglycine in which the acyl group is specified as isovaleryl.
