Mannose Associated Serine Protease 2 (MASP2) Antibody – Rabbit Polyclonal

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]

MASP2: 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. Defects in MASP2 are the cause of MASP2 deficiency (MASPD). MASPD is a disorder that results in autoimmune manifestations, recurrent severe infections, and chronic inflammatory disease. Belongs to the peptidase S1 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 3.4.21.104; Protease; Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 1p36.22

Cellular Component: extracellular region; extracellular space

Molecular Function: calcium ion binding; calcium-dependent protein binding; complement component C4b binding; peptidase activity; protein binding; serine-type endopeptidase activity

Biological Process: complement activation; complement activation, classical pathway; complement activation, lectin pathway; proteolysis

Disease: Masp2 Deficiency