NEET MDS

The Power Course

Warner Syndrome

Instructions

A 42-year-old present with premature aging. He is diagnosed as Werner syndrome. The most likely mechanism is.



Choices

1 ) Decreased lipid peroxidation
2 ) Increased advanced glycation end products
3 ) Short telomere with damaged DNA and loss of helicase
4 ) Increased length of telomere

Explanation


Werner syndrome

This is an autosomal recessive disease. These patients present with premature aging. Up to teenage, patients are normal. There is no growth spurt of teens. from the beginning of twenties signs of the early aging manifest. They are hoarse voice, thin course skin, thin limbs, abdominal obesity (fat deposition), early atherosclerosis, and prematurely graying hair. These patients may suffer from the early onset of cancer and complication of diabetes and atherosclerosis. Most of them die in the early fifties.

Genetics

Inability to repair damaged DNA, the complication of abnormal Werner protein produced by the mutated Werner gene. The Werner protein functions as a type of enzyme called a helicase and exonuclease. telomere shortening also present


Answer

Short telomere with damaged DNA and loss of helicase

Last Modified : 17-Jan-2020

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