CBS - Cystathionine beta-synthase - human protein (Function)
 
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CBS »  Cystathionine beta-synthase   [ EC 4.2.1.22 ]
 
Protein also known as:  Serine sulfhydrase.
Gene name:  CBS
Entry whose protein(s) existence is based on evidence at protein level
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1 167 2
GENE REF ISO

Function

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Overview 
Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury (By similarity).  
  • CuratedUniProtKB
GO molecular function 
Adenyl nucleotide bindingdefinition[GO:0030554] silver  
  • IEAInterPro 2 GO
Cystathionine beta-synthase activitydefinition[GO:0004122]  
3
  • IDAUniProtKB
Enzyme bindingdefinition[GO:0019899]  
1
  • IPIUniProtKB
Heme bindingdefinition[GO:0020037]  
2
  • IMPUniProtKB
Identical protein bindingdefinition[GO:0042802]  
3
  • IPIIntAct
Metal ion bindingdefinition[GO:0046872]  
  • IEAUniProtKB KW
Modified amino acid bindingdefinition[GO:0072341]  
1
  • IDAUniProtKB
Protein bindingdefinition[GO:0005515]  
1
  • IPIUniProtKB
Protein homodimerization activitydefinition[GO:0042803]  
1
  • IDAUniProtKB
Pyridoxal phosphate bindingdefinition[GO:0030170]  
2
  • IDAUniProtKB
Ubiquitin protein ligase bindingdefinition[GO:0031625]  
1
  • IPIUniProtKB
GO biological process 
Blood vessel remodelingdefinition[GO:0001974] silver  
  • IEAOrtholog Compara
Cartilage development involved in endochondral bone morphogenesisdefinition[GO:0060351] silver  
  • IEAOrtholog Compara
Cellular response to hypoxiadefinition[GO:0071456] silver  
  • IEAOrtholog Compara
Cerebellum morphogenesisdefinition[GO:0021587] silver  
  • IEAOrtholog Compara
Cysteine biosynthetic process from serinedefinition[GO:0006535] silver  
  • IEAInterPro 2 GO
Cysteine biosynthetic process via cystathioninedefinition[GO:0019343] silver  
  • IEAInterPro 2 GO
Endochondral ossificationdefinition[GO:0001958] silver  
  • IEAOrtholog Compara
Homocysteine catabolic processdefinition[GO:0043418]  
1
  • IDAUniProtKB
Homocysteine metabolic processdefinition[GO:0050667]  
2
  • IDAUniProtKB
Hydrogen sulfide biosynthetic processdefinition[GO:0070814]  
1
  • IDAUniProtKB
  • ISSOrtholog Curator
L-cysteine catabolic processdefinition[GO:0019448]  
1
  • IDAUniProtKB
L-serine catabolic processdefinition[GO:0006565]  
1
  • IDAUniProtKB
L-serine metabolic processdefinition[GO:0006563]  
1
  • IDAUniProtKB
Maternal process involved in female pregnancydefinition[GO:0060135] silver  
  • IEAOrtholog Compara
Negative regulation of apoptotic processdefinition[GO:0043066] silver  
  • IEAOrtholog Compara
Regulation of blood vessel sizedefinition[GO:0050880] silver  
  • IEAOrtholog Compara
Regulation of cGMP metabolic processdefinition[GO:0030823] silver  
  • IEAOrtholog Compara
Regulation of JUN kinase activitydefinition[GO:0043506] silver  
  • IEAOrtholog Compara
Response to folic aciddefinition[GO:0051593] silver  
  • IEAOrtholog Compara
Superoxide metabolic processdefinition[GO:0006801] silver  
  • IEAOrtholog Compara
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 4.2.1.22: L-serine + L-homocysteine L-cystathionine + H(2)O.  
  • CuratedUniProtKB
It requires the following cofactor
Pyridoxal phosphate.  
  • CuratedUniProtKB
It is regulated in the following manner
Allosterically activated by adenosyl-methionine (AdoMet).  
  • CuratedUniProtKB
Pathways 
This protein is involved in the following pathway
According to KEGG, this protein belongs to the following pathways:
Cysteine and methionine metabolism  hsa00270+875  
Glycine, serine and threonine metabolism  hsa00260+875  
Metabolic pathways  hsa01100+875  
According to Reactome, this protein belongs to the following pathway:
Cysteine formation from homocysteine  REACT_115589  
 
More information is available from:
 

Keywords

Biological process 
Amino-acid biosynthesis  definition   [KW-0028]
Cysteine biosynthesis  definition   [KW-0198]
Molecular function 
Lyase  definition   [KW-0456]
Technical term 
Allosteric enzyme  definition   [KW-0021]
Reference proteome  definition   [KW-1185]
 

Further external links

Enzyme and pathway databases
SABIO-RK: P35520
Other
GeneWiki: Cystathionine_beta_synthase
GenomeRNAi: 875
PRO: PR:P35520
Chemistry
GuidetoPHARMACOLOGY: 1443