InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: ADAMTS13

Summary

InnateDB Protein

IDBP-91332.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

ADAMTS13

Protein Name

ADAM metallopeptidase with thrombospondin type 1 motif, 13

Synonyms

ADAM-TS13; ADAMTS-13; C9orf8; vWF-CP; VWFCP;

Species

Homo sapiens

Ensembl Protein

ENSP00000360997

InnateDB Gene

IDBG-91330 (ADAMTS13)

Protein Structure

UniProt Annotation

Function

Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation. {ECO:0000269PubMed:19880749}.

Subcellular Localization

Secreted {ECO:0000269PubMed:12791682}. Note=Secretion enhanced by O-fucosylation of TSP type-1 repeats.

Disease Associations

Thrombotic thrombocytopenic purpura congenital (TTP) [MIM:274150]: A hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever. recessive. {ECO:0000269PubMed:11586351, ECO:0000269PubMed:12181489, ECO:0000269PubMed:12393505, ECO:0000269PubMed:12614216, ECO:0000269PubMed:12753286, ECO:0000269PubMed:14512317, ECO:0000269PubMed:14563640, ECO:0000269PubMed:15009458, ECO:0000269PubMed:15126318, ECO:0000269PubMed:15327386, ECO:0000269PubMed:16449289, ECO:0000269PubMed:16453338, ECO:0000269PubMed:16796708, ECO:0000269PubMed:16807643, ECO:0000269PubMed:17003922, ECO:0000269PubMed:18443791, ECO:0000269PubMed:19055667, ECO:0000269PubMed:19116307, ECO:0000269PubMed:22075512}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Plasma. Expressed primarily in liver. {ECO:0000269PubMed:11574066}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 3 experimentally validated interaction(s) in this database.

Experimentally validated
Total	3 [view]
Protein-Protein	3 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004175	endopeptidase activity
GO:0004222	metalloendopeptidase activity
GO:0005178	integrin binding
GO:0005509	calcium ion binding
GO:0005515	protein binding
GO:0008233	peptidase activity
GO:0008237	metallopeptidase activity
GO:0008270	zinc ion binding

Biological Process

GO:0006508	proteolysis
GO:0007160	cell-matrix adhesion
GO:0007229	integrin-mediated signaling pathway
GO:0009100	glycoprotein metabolic process
GO:0009636	response to toxic substance
GO:0016485	protein processing
GO:0030168	platelet activation
GO:0034341	response to interferon-gamma
GO:0034612	response to tumor necrosis factor
GO:0043171	peptide catabolic process
GO:0070670	response to interleukin-4

Cellular Component

GO:0005578	proteinaceous extracellular matrix
GO:0005615	extracellular space
GO:0009986	cell surface

Protein Structure and Domains

PDB ID

InterPro

IPR000859 CUB domain
IPR000884 Thrombospondin, type 1 repeat
IPR001590 Peptidase M12B, ADAM/reprolysin
IPR006586 ADAM, cysteine-rich
IPR013273 Peptidase M12B, ADAM-TS

PFAM

PF00431
PF00090
PF01421
PF08516

PRINTS

PR01857

PIRSF

SMART

SM00042
SM00209
SM00608

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt