ADAMTS13 - A disintegrin and metalloproteinase with thrombospondin motifs 13 - human protein (Function)
 
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ADAMTS13 »  A disintegrin and metalloproteinase with thrombospondin motifs 13   [ EC 3.4.24.87 ]  (ADAM-TS13)
 
Protein also known as:  von Willebrand factor-cleaving protease (vWF-CP).
Gene name:  ADAMTS13
Entry whose protein(s) existence is based on evidence at protein level
extend overview
1 69 4
GENE REF ISO

Function

 show evidences
Overview 
Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation.  
  • CuratedUniProtKB
GO molecular function 
Calcium ion bindingdefinition[GO:0005509]  
1
  • TASUniProtKB
Integrin bindingdefinition[GO:0005178]  
1
  • TASUniProtKB
Metalloendopeptidase activitydefinition[GO:0004222] silver  
  • IEAInterPro 2 GO
Metallopeptidase activitydefinition[GO:0008237]  
1
  • TASUniProtKB
Protein bindingdefinition[GO:0005515]  
2
  • IPIIntAct
  • IPIUniProtKB
Zinc ion bindingdefinition[GO:0008270]  
1
  • TASUniProtKB
GO biological process 
Cell-matrix adhesiondefinition[GO:0007160]  
1
  • NASUniProtKB
Glycoprotein metabolic processdefinition[GO:0009100]  
1
  • NASUniProtKB
Integrin-mediated signaling pathwaydefinition[GO:0007229]  
1
  • NASUniProtKB
Peptide catabolic processdefinition[GO:0043171]  
1
  • IDAUniProtKB
Platelet activationdefinition[GO:0030168]  
1
  • NASUniProtKB
Protein processingdefinition[GO:0016485]  
1
  • TASUniProtKB
Proteolysisdefinition[GO:0006508]  
1
  • IDAUniProtKB
Response to interferon-gammadefinition[GO:0034341] silver  
  • IEAOrtholog Compara
Response to interleukin-4definition[GO:0070670] silver  
  • IEAOrtholog Compara
Response to tumor necrosis factordefinition[GO:0034612] silver  
  • IEAOrtholog Compara
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reactions
Cleaves the vWF at the 842-Tyr-|-Met-843 in the A2 domain of the vWF subunit.  
  • CuratedUniProtKB
EC 3.4.24.87: The enzyme cleaves the von Willebrand factor at bond Tyr(842)-|-Met(843) within the A2 domain.  
  • CuratedUniProtKB
It requires the following cofactors
Binds 1 zinc ion per subunit (By similarity).  
  • CuratedUniProtKB
Binds 4 calcium ions (Potential).  
  • CuratedUniProtKB
It is regulated in the following manner
Zinc and calcium ions cooperatively modulate enzyme activity. The cleavage of the pro-domain is not required for protease activity. Dependence on calcium for proteolytic activity is mediated by the high affinity site.  
3
  • CuratedUniProtKB
 
More information is available from:
Pathways 
According to Reactome, this protein belongs to the following pathway:
O-glycosylation of TSR domain-containing proteins  REACT_200626  
 

Keywords

Biological process 
Blood coagulation  definition   [KW-0094]
Hemostasis  definition   [KW-0356]
Molecular function 
Hydrolase  definition   [KW-0378]
Metalloprotease  definition   [KW-0482]
Protease  definition   [KW-0645]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

GeneWiki: ADAMTS13
GenomeRNAi: 11093
PRO: PR:Q76LX8