N-acetyl-L-Isoleucine

N-acetyl-L-Isoleucine

CAT N°: 35545
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From 77.00 65.45

N-acetyl-L-Isoleucine is an acetylated form of L-isoleucine. Urine levels of N-acetyl-L-isoleucine are increased in patients with maple syrup urine disease (MSUD), an inborn error of metabolism characterized by a deficiency in the branched-chain ?-ketoacid dehydrogenase complex, the complex that catalyzes the degradation of branched-chain amino acids, leading to the accumulation of branched-chain amino acids in the plasma and urine, a maple syrup-like odor in the urine, and neurological impairments.{57731}

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