MASP2

This gene encodes a member of the peptidase S1 family of serine proteases. The encoded preproprotein is proteolytically processed to generate A and B chains that heterodimerize to form the mature protease. This protease cleaves complement components C2 and C4 in order to generate C3 convertase in the lectin pathway of the complement system. The encoded protease also plays a role in the coagulation cascade through cleavage of prothrombin to form thrombin. Myocardial infarction and acute stroke patients exhibit reduced serum concentrations of the encoded protein. Alternative splicing results in multiple transcript variants, at least one of which encodes an isoform that is proteolytically processed. [provided by RefSeq, Feb 2016]

Full Name

Mannan Binding Lectin Serine Peptidase 2

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.

Biological Process

Cell surface pattern recognition receptor signaling pathway Source: ComplexPortal
Complement activation, classical pathway Source: UniProtKB-KW
Complement activation, lectin pathway Source: ComplexPortal
Positive regulation of opsonization Source: ComplexPortal
Proteolysis Source: ComplexPortal

Cellular Location

Secreted

Involvement in disease

MASP2 deficiency (MASPD):
A disorder that results in autoimmune manifestations, recurrent severe infections, and chronic inflammatory disease.

PTM

The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.
Activated by cleavage after Arg-444. The uncleaved zymogen is inactive towards synthetic substrates, but has sufficient activity to effect autocatalytic cleavage.