Congenital lactic acidosis occur due to the deficiency of which of the following enzyme?
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
Transketolase
Pyruvate dehydrogenase enzyme complex
Pyruvate kinase
Pyruvate decarboxylase
Metabolism of carbohydrate