Diseases and Disorders, Diseases of Amino Acid and Organic Acid Metabolism, Disorders of Fatty Acid Metabolism
Last updated: June 2, 2026 Introduction to Organic Acidurias Organic acidurias (OAD; also identified as organic acidemias) refers to a biochemically defined group of inherited metabolic diseases resulting from failure to metabolize various organic acids. The OAD can...
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...