Diseases and Disorders, Diseases of Amino Acid and Organic Acid Metabolism
Last Updated December 14, 2025 Introduction to Branched-Chain Amino Acid Metabolism Disorders The branched-chain amino acids are leucine, isoleucine, and valine. The catabolism of all three branched-chain amino acids (BCAA) occurs in most cells but the highest rates...
Diseases and Disorders, Diseases of Amino Acid and Organic Acid Metabolism
Last updated: October 28, 2025 Introduction to Maple Syrup Urine Disease: MSUD Maple syrup urine disease (MSUD), also called branched-chain aminoaciduria, is so called because the urine of affected individuals smells like maple syrup or burnt sugar. The cause of the...