| cystathionine |
(cys
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| cystathionine β-synthase |
[EC 4.2.1.22] an enzyme of the lyase class that catalyzes the condensation of serine and homocysteine to form cystathionine as a step in the catabolism of methionine. It is a pyridoxal-phosphate protein, found in the mammalian liver.
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| cystathionine β-synthase deficiency |
an autosomal recessive aminoacidopathy characterized by homocystinuria accompanied by hypermethioninemia. Clinical abnormalities occur primarily in the eye and the skeletal, nervous, and vascular systems; ectopia lentis, osteoporosis, mental retardation, and thrombosis are the most common manifestations. In older literature, the disorder is sometimes called homocystinuria (q.v.).
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| cystathionine γ-lyase |
[EC 4.4.1.1] an enzyme of the lyase class that catalyzes the cleavage of cystathionine to cysteine, α-ketoglutarate, and ammonia as a step in the metabolism of methionine. It is a pyridoxal-phosphate protein and the reaction occurs in the liver. Deficiency of the enzyme, an autosomal recessive trait, results in cystathioninuria. Called also γ-cystathionase.
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