Lack of beta-oxidation of fatty acid leads to?: (A) Oxidation of branched chain fatty acids, (B) Phytanic acid accumulation, (C) Dicarboxylic acid accumulation, (D) Formation of propionic acid
Answer is C. Lack of beta-oxidation of fatty acids leads to the accumulation of dicarboxylic acids. Dicarboxylic aciduria is caused by a lack of mitochondrial medium-chain acylCoA dehydrogenase. It is characterized by 1. Excretion of C -C o-dicarboxylic acids in the urine. 2.Nonketotic hypoglycemia Reference: Harper's Illustrated Biochemistry 31st edition Pg 215