Activating Compound | Comment | Organism | Structure |
---|---|---|---|
S-adenosyl-L-methionine | an allosteric activator | Homo sapiens |
Application | Comment | Organism |
---|---|---|
diagnostics | evaluation of plasma cystathionine beta-synthase activity in cystathionine beta-synthase-deficient patients for utilization of the assay in diagnosis and study of cystathionine beta-synthase deficiency | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
cytosol | - |
Homo sapiens | 5829 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
L-serine + L-homocysteine | Homo sapiens | - |
L-cystathionine + H2O | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
kidney | - |
Homo sapiens | - |
liver | - |
Homo sapiens | - |
muscle | - |
Homo sapiens | - |
pancreas | - |
Homo sapiens | - |
plasma | cystathionine beta-synthase activities in wild-type individuals, and in hetero-, and homozygote cystathionine beta-synthase mutants, overview | Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
L-serine + L-homocysteine | - |
Homo sapiens | L-cystathionine + H2O | - |
? |
Synonyms | Comment | Organism |
---|---|---|
CBS | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
pyridoxal 5'-phosphate | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | cystathionine beta-synthase deficiency is a well-known genetic disease affecting the first step in the conversion of homocysteine to cysteine and ultimately to inorganic sulfur. The disease occurs in a mild and a severe form, phenotypes, overview. Cystathionine beta-synthase activities in wild-type individuals, and in hetero-, and homozygote cystathionine beta-synthase mutants, overview | Homo sapiens |