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Biochemistry Metabolism of carbohydrate 0134bde6

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
High-Yield Explanation
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

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