Congenital lactic acidosis occur due to the deficiency of which of the following enzyme?: (A) Transketolase, (B) Pyruvate dehydrogenase enzyme complex, (C) Pyruvate kinase, (D) Pyruvate decarboxylase
Answer is B. Inherited pyruvate dehydrogenase deficiency, presents with lactic acidosis. Due to lack of pyruvate dehydrogenase,there will be lack of Acetyl co A production and pyruvate to lactate conversion occurs leading to accumulation of lactic acid Causes of lactic acidosis: 1. Tissue hypoxia 2. Mitochondrial disorders 3. Deficiency of: PDH Enzyme TCA cycle enzymes Vit B1 4. Poisoning : Cyanide, Mercury, Arsenite Reference: Harper's Illustrated Biochemistry 30th Edition Page no: 174