InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: MASP2

Summary

InnateDB Protein

IDBP-227136.5

Last Modified

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

Gene Symbol

MASP2

Protein Name

mannan-binding lectin serine peptidase 2

Synonyms

MAP19; MASP-2; MASP1P1; sMAP;

Species

Homo sapiens

Ensembl Protein

ENSP00000383690

InnateDB Gene

IDBG-89176 (MASP2)

Protein Structure

UniProt Annotation

Function

Serum protease that plays an important role in the activation of the complement system via mannose-binding lectin. After activation by auto-catalytic cleavage it cleaves C2 and C4, leading to their activation and to the formation of C3 convertase. {ECO:0000269PubMed:10946292}.

Subcellular Localization

Secreted.

Disease Associations

MASP2 deficiency (MASPD) [MIM:613791]: A disorder that results in autoimmune manifestations, recurrent severe infections, and chronic inflammatory disease. {ECO:0000269PubMed:12904520, ECO:0000269PubMed:17252003}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Plasma.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 13 experimentally validated interaction(s) in this database.

Experimentally validated
Total	13 [view]
Protein-Protein	13 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0001855	complement component C4b binding
GO:0003824	catalytic activity
GO:0004252	serine-type endopeptidase activity
GO:0005509	calcium ion binding
GO:0005515	protein binding
GO:0008233	peptidase activity
GO:0048306	calcium-dependent protein binding

Biological Process

GO:0001867	complement activation, lectin pathway
GO:0006508	proteolysis
GO:0006956	complement activation
GO:0006958	complement activation, classical pathway
GO:0045087	innate immune response (InnateDB)

Cellular Component

GO:0005576	extracellular region
GO:0070062	extracellular vesicular exosome

Protein Structure and Domains

PDB ID

InterPro

IPR000436 Sushi/SCR/CCP
IPR000742 Epidermal growth factor-like domain
IPR000859 CUB domain
IPR001254 Peptidase S1
IPR001314 Peptidase S1A, chymotrypsin-type
IPR001881 EGF-like calcium-binding domain
IPR009003 Trypsin-like cysteine/serine peptidase domain

PFAM

PF00084
PF00008
PF00431
PF00089
PF07645

PRINTS

PR00722

PIRSF

SMART

SM00032
SM00181
SM00042
SM00020
SM00179

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt