cystathionine beta-synthase

(redirected from cystathionine b-synthase)

cys·ta·thi·o·nine β-syn·thase

(sis'tă-thī'ō-nēn sin'thās),
An enzyme catalyzing the reversible hydrolysis of l-cystathionine to l-serine and l-homocysteine. A step in cysteine biosynthesis and in methionine catabolism. A deficiency of this enzyme leads to vascular thrombosis, dislocation of ocular lens, and abnormal development.
See also: cystathionine γ-synthase.

cystathionine β-synthase

/cys·ta·thi·o·nine β-syn·thase/ (sin´thās) a pyridoxal phosphate–containing lyase that catalyzes a step in the catabolism of methionine; deficiency occurs in an aminoacidopathy characterized by homocystinuria, elevated blood methionine levels, and abnormalities in the eye and the skeletal, nervous, and vascular systems.
References in periodicals archive ?
Homocysteine, a sulfur containing amino-acid is either methylated to form methionine and then into S-adenosylmethionine (SAM) or trans-sulfurated into cystathionine in the presence of cystathionine b-synthase (CBS).
1,25-dihydroxyvitamin D3 influences cellular homocysteine levels in murine in pre-osteoblastic MC3T3-E1 cells by direct regulation of cystathionine b-synthase.
Cystathionine b-synthase Methionine synthase Methylenetetrahydrofolate reductase
A yeast assay for functional detection of mutations in the human cystathionine b-synthase gene.
HT-CBS, heterozygotes for cystathionine b-synthase deficiency.