Which is not pyridoxine dependenta) Homocystinuria b) Maple syrup urine diseasec) Oxaluriad) Xanthinuria
Correct Answer: bd
Description: Pyridoxal phosphate-dependent conditions are homocystinuria, cystathioninuria, oxaluria and xanthurenic aciduria.
Don't get confused option d (xanthinuria) with xanthurenic aciduria. Both are different conditions
In xanthurenic aciduria, there is excretion of xanthurenic acid (xanturenate), an alternative metabolite of tryptophan metabolism. This is pyridoxine-dependent.
In xanthinuria, there is excessive excretion of xanthine in the urine. This is due to deficiency of enzyme xanthine oxidase and it is not pyridoxine responsive.
About option b
Maple syrup urine disease is due to defective branched chain u-ketoacid dehydrogenase enzyme, and it may be associated with thiamine (vitamin B1) deficiency.
Category:
Biochemistry
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