Pronunciation: sis'ta-thi'o-nen li'as
Definition: A liver enzyme, requiring pyridoxal phosphate as coenzyme, which catalyzes the hydrolysis of l-cystathionine to l-cysteine and 2-ketobutyrate, releasing NH3; also catalyzes formation of 2-ketobutyrate from l-homoserine, of pyruvate (and NH3 and H2S) from l-cysteine, and of thiocysteine, pyruvate, and NH3 from cystine. A deficiency of this enzyme results in cystathioninuria. It catalyzes a step in methionine catabolism and in cysteine biosynthesis.
See Also: cystathionine ß-lyase
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