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Cystathionine-β-synthase, also known as CBS, is an enzyme (EC 4.2.1.22) that in humans is encoded by the CBS gene. It catalyzes the first step of the transsulfuration pathway , from homocysteine to cystathionine : [ 5 ]
In enzymology, a cystathionine gamma-synthase (EC 2.5.1.48) is an enzyme that catalyzes the formation of cystathionine from cysteine and an activated derivative of homoserine, e.g.: O 4 -succinyl-L-homoserine + L-cysteine {\displaystyle \longrightarrow } L-cystathionine + succinate
Cystathionine is an intermediate in the synthesis of cysteine from homocysteine. It is produced by the transsulfuration pathway and is converted into cysteine by cystathionine gamma-lyase (CTH). Biosynthetically, cystathionine is generated from homocysteine and serine by cystathionine beta synthase (upper reaction in the diagram below).
Homocystinuria (HCU) [2] is an inherited disorder of the metabolism of the amino acid methionine due to a deficiency of cystathionine beta synthase or methionine synthase. [3] It is an inherited autosomal recessive trait, which means a child needs to inherit a copy of the defective gene from both parents to be affected.
It is characterized by an abnormal accumulation of plasma cystathionine leading to excess cystathionine in the urine. Hereditary cystathioninuria is associated with the reduced activity of the enzyme cystathionine gamma-lyase. [2] It is considered a biochemical anomaly. This is because it associated with a wide range of diseases and its ...
Other members include cystathionine γ synthase, cystathionine β lyase, and methionine γ lyase. [8] It is also a member of the broader aspartate aminotransferase family. [1] [8] Like many other PLP-dependent enzymes, cystathionine γ-lyase is a tetramer with D2 symmetry. [8] Pyridoxal phosphate is bound in the active site by Lys 212. [2]
Cystathionine beta-lyase (EC 4.4.1.8), also commonly referred to as CBL or β-cystathionase, is an enzyme that primarily catalyzes the following α,β-elimination reaction [1] Reaction catalyzed by cystathionine beta-lyase. Thus, the substrate of this enzyme is L-cystathionine, whereas its 3 products are homocysteine, pyruvate, and ammonia. [2 ...
Cysteine synthesis: Cystathionine beta synthase catalyzes the upper reaction and cystathionine gamma-lyase catalyzes the lower reaction. In animals, biosynthesis begins with the amino acid serine. The sulfur is derived from methionine, which is converted to homocysteine through the intermediate S-adenosylmethionine.