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Literature summary for 4.2.1.22 extracted from

  • Casique, L.; Kabil, O.; Banerjee, R.; Martinez, J.C.; De Lucca, M.
    Characterization of two pathogenic mutations in cystathionine beta-synthase: different intracellular locations for wild-type and mutant proteins (2013), Gene, 531, 117-124.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 4.2.1.22

Activating Compound

Activating Compound Comment Organism Structure
S-adenosyl-L-methionine
-
 Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
expressed in Escherichia coli Homo sapiens

Protein Variants

Protein Variants Comment Organism
D234N the mutation is associated with Venezuelan homocystinuria responsive to vitamin B6. The mutant shows 43% activity compared to the wild type enzyme Homo sapiens
T87N the mutation is associated with Venezuelan homocystinuria nonresponsive to vitamin B6. The mutant shows 3.5% activity compared to the wild type enzyme Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
L-serine + L-homocysteine Homo sapiens
-
 L-cystathionine + H2O
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens P35520
-
-

Substrates and Products (Substrate)

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

Subunits

Subunits Comment Organism
tetramer
-
 Homo sapiens

Synonyms

Synonyms Comment Organism
CBS
-
 Homo sapiens

Cofactor

Cofactor Comment Organism Structure
heme
-
 Homo sapiens
pyridoxal 5'-phosphate dependent on Homo sapiens