Cystathionine beta-synthase

spinqualitylabelsall models Human cystathionineβ-synthase complex with heme, PLP and SAM (PDB id 4pcu) Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Function Cystathionine β-synthase (CBS) catalyzes the PLP-dependent interconversion of cysteine and homocysteine. In bacterial systems CBS catalyzes the transfer of thiol from cysteine to homocysteine and in mammalian systems from homocysteine to cysteine.. Disease CBS deficiency causes homocysteinuria which affects the eye, skeletal system, vascular system and the CNS. Mutations in Ala114, Ile278, G305 and G307 were found to be involved in homocysteinuria. Structural highlights The structure of the complex of CBS with heme, PLP and SAM shows the N-terminal domain which binds the heme cofactor interacting with Cys52 and His65. The catalytic core interacts with the PLP cofactor at Lys119 and a C-terminal regulatory domain. 3D structures of cystathionine β-synthase Cystathionine β-synthase 3D structures

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Michal Harel