Diseases and Disorders, Diseases of Amino Acid and Organic Acid Metabolism
Last Updated May 28, 2026 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 of...
Diseases and Disorders, Diseases of Amino Acid and Organic Acid Metabolism
Last updated: May 31, 2026 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 odor...