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Biochemistry

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Vitamins, Harmones and Hemoglobin

Question
8 out of 82
 

Which is not Pyridoxine dependent



A Oxaluria

B Homocystinuria

C Maple syrup urine disease

D Xanthinuric aciduria

Ans. C Maple syrup urine disease

a. Vit. B6 (Pyridoxine) responsive syndrome (disease) : are

1. Infantile convulsions

2. Cystathionuria (defect in cystathioninase)

3. Homocystinuria (cystathionine synthase)

b. Pyridoxal — phosphate (Schiff base) is active form of vit. B6

i. Acts as co-enzyme for — Amino transferases (Transaminases)

ii. Acts as co-enzyme for Decarboxylase and cofactor of glycogen phosphorylase

iii. Acts as co-enzyme for Deaminases

iv. As Co-enzyme for Kynureninase: an enzyme for pathway of Niacin synthesis, from tryptophan Thus in vit. B6 deficiency, Niacin synthesis does not taken place. In vit-B6 deficiency, Kynurenine and 3-OH kynurenine —

v. are converted into xanthurenic acid in extrahepatic tissues, which is excreted in urine. So, “Xanthurenic acid index” is a reliable criteria for Vit. B6 deficiency.

Hence, Xanthuria aciduria is vit B6 responsive

vi. Oxaluria vit B6 deficiency has been observed to produce oxaluria in experimental animals:

hence oxaluria is B6 responsive.

c. Vit. B6 important in steroid hormone action i.e. terminate the action of hormones therefore its deficiency causes led sensitivity to steroid hormone action result hormone dependent cancer of breast, uterus and prostrate.

Vitamins, Harmones and Hemoglobin Flashcard List

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