Which is not Pyridoxine-dependent?
Correct Answer: Maple syrup urine disease
Description: Deficiency of Vitamin B6 (Pyridoxine) leads to:- Peripheral neuropathy Convulsions: Due to decreased synthesis of GABA Microcytic hypochromic Anemia: Due to decreased heme synthesis Pellagra due to defective niacin synthesis Oxaluria: Due to defective Alanine: Glyoxylate Amino Transferase. Glyoxylate conveed to Oxalic acid Homocystinuria: Due to defective Cystathionine Beta-Synthase Xanthurenic Aciduria: Due to defective Kynureninase Cardiovascular risks: Because of homocysteinemia Maple syrup urine disease is the defect of deficient decarboxylation of branched-chain keto acids. Decarboxylation of the BKA requires thiamine. Reference: DM.VASUDEVAN.TEXTBOOK 8th EDITION page no ;472
Category:
Biochemistry
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