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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 α-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.1
WARNING This product is not for human or veterinary use.
1. Urinary excretion of N-