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The modular domain structure of human CBS. The human CBS consists of three domains: the N-terminal heme-binding domain with Cys-52 and His-65 as axial ligands of heme b, the highly conserved catalytic core with the ϵ-amino group of Lys-119 binding the carbonyl group of PLP, and the C-terminal AdoMet-binding regulatory domain with a tandem of CBS domains. The positions of the CBS mutations characterized in this study are also shown.
