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Q. What is Homocystinuria?
Homocystinuria may result from errors of methionine metabolism. This produces defects in the function of cystathionme b synthase or 5-mcthyltctrahydrofolate homocysteine methyltransferase.
The most common form of hornocystinuria is caused by a deficiency of the enzyme cystathionine b synthase. This enzyme is essential for the conversion of homocysteine to cystathionine. Severely impaired enzyme function produces accumulation of plasma homocysteine and methionine and decreased cysteine in cells. Large amounts of homocystine are excreted in the urine.
Indications for Surgery : The normal mitral valve area is 4-5 cm 2 . Usually symptoms appear when the valve area has become less than 2.5 cm2. Symptoms are present at rest when t
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A disulfide bond can form between any two amino acids that have sulfur in their side-chain.
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