CBS (D8F2P) Rabbit mAb #14782

Cystathionine beta-synthase (CBS) is a key enzyme involved in sulfur amino acid metabolism because it catalyzes the formation of cystathionine from serine and homocysteine (1,2). The CBS protein contains a heme-binding domain that modulates enzyme activity by sensing redox changes or carbon monoxide binding (1). S-adenosylmethionine binds the carboxyl-terminal CBS domain to allosterically regulate CBS catalytic activity (3,4). In addition to catalyzing cystathionine formation, CBS also catalyzes the generation of hydrogen sulfide, a neuromodulator in the brain, through alternative reactions (5,6). Mutations in the corresponding CBS gene result in homocystinuria, an autosomal recessive disorder characterized by abnormal sulfur metabolism, mental retardation, eye anomalies, and vascular disease (7).