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Literature summary extracted from

  • Gupta, S.; Wang, L.; Hua, X.; Krijt, J.; Kozich, V.; Kruger, W.D.
    Cystathionine beta-synthase p.S466L mutation causes hyperhomocysteinemia in mice (2008), Hum. Mutat., 29, 1048-1054.
    View publication on PubMedView publication on EuropePMC

Activating Compound

EC Number Activating Compound Comment Organism Structure
4.2.1.22 S-adenosyl-L-methionine about 4fold increase of specific activity in the presence of 0.25 mM S-adenosyl-L-methionine Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
4.2.1.22 wild type enzyme is expressed in Mus musculus and mutant enzyme S466L is expressed in Saccharomyces cerevisiae strain WY35 and in Mus musculus Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
4.2.1.22 S466L the mutation causes hyperhomocysteinemia by affecting both the steady state level of CBS protein and by reducing the efficiency of the enzyme in vivo, S466L is enzymatically active, forms tetramers, and lacks S-adenosyl-L-methionine inducibility Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
4.2.1.22 0.77
-
L-serine mutant enzyme S466L, in the presence of 0.25 mM S-adenosyl-L-methionine Homo sapiens
4.2.1.22 0.78
-
L-serine wild type enzyme, in the presence of 0.25 mM S-adenosyl-L-methionine Homo sapiens
4.2.1.22 1.1
-
L-homocysteine mutant enzyme S466L, in the presence of 0.25 mM S-adenosyl-L-methionine Homo sapiens
4.2.1.22 2.5
-
L-homocysteine wild type enzyme, in the presence of 0.25 mM S-adenosyl-L-methionine Homo sapiens

Organism

EC Number Organism UniProt Comment Textmining
4.2.1.22 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
4.2.1.22 brain
-
Homo sapiens
-
4.2.1.22 kidney
-
Homo sapiens
-
4.2.1.22 liver highest activity Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4.2.1.22 L-serine + L-homocysteine
-
Homo sapiens L-cystathionine + H2O
-
?

Synonyms

EC Number Synonyms Comment Organism
4.2.1.22 CBS
-
Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
4.2.1.22 pyridoxal 5'-phosphate
-
Homo sapiens