L-Alloisoleucine

L-Alloisoleucine

CAT N°: 34904
Price:

From 89.00 75.65

L-Alloisoleucine is a branched-chain amino acid and diastereomer of L-isoleucine.{61892} It is formed from L-isoleucine by transamination.{61893} Plasma L-alloisoleucine levels 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.{61892}

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