InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: C2

Summary

InnateDB Protein

IDBP-373396.5

Last Modified

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

Gene Symbol

Protein Name

complement component 2

Synonyms

ARMD14; CO2;

Species

Homo sapiens

Ensembl Protein

ENSP00000392322

InnateDB Gene

IDBG-124824 (C2)

Protein Structure

UniProt Annotation

Function

Component C2 which is part of the classical pathway of the complement system is cleaved by activated factor C1 into two fragments: C2b and C2a. C2a, a serine protease, then combines with complement factor C4b to generate the C3 or C5 convertase.

Subcellular Localization

Secreted.

Disease Associations

Complement component 2 deficiency (C2D) [MIM:217000]: A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus erythematosus. Skin and joint manifestations are common and renal disease is relatively rare. Patients with complement component 2 deficiency are also reported to have recurrent invasive infections. {ECO:0000269PubMed:8621452, ECO:0000269PubMed:9670930}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 8 experimentally validated interaction(s) in this database.

Experimentally validated
Total	8 [view]
Protein-Protein	5 [view]
Protein-DNA	3 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003824	catalytic activity
GO:0004252	serine-type endopeptidase activity

Biological Process

GO:0006508	proteolysis
GO:0006956	complement activation
GO:0006958	complement activation, classical pathway
GO:0030449	regulation of complement activation
GO:0045087	innate immune response (InnateDB)
GO:2000427	positive regulation of apoptotic cell clearance

Cellular Component

GO:0005576	extracellular region
GO:0005615	extracellular space

Protein Structure and Domains

PDB ID

InterPro

IPR000436 Sushi/SCR/CCP
IPR001254 Peptidase S1
IPR001314 Peptidase S1A, chymotrypsin-type
IPR002035 von Willebrand factor, type A
IPR009003 Trypsin-like cysteine/serine peptidase domain

PFAM

PF00084
PF00089
PF00092

PRINTS

PR00722

PIRSF

SMART

SM00032
SM00020
SM00327

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt