MASP-2 Rabbit Polyclonal Antibody

Mannan binding lectin serine peptidase 2 (MASP2) Homo sapiens 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], catalytic activity:Selective cleavage after Arg-223 in complement component C2 (-Ser-Leu-Gly-Arg-|-Lys-Ile-Gln-Ile) and after Arg-76 in complement component C4 (-Gly-Leu-Gln-Arg-|-Ala-Leu-Glu-Ile) ., disease:Genetic variation in MASP2 is the cause of MASP2 deficiency [MIM:605102]. It is associated with susceptibility to infections and with the development of immunologic disease., 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., miscellaneous:Dimerization and MBL2 binding requires calcium ions., online information:MASP2 mutation db, PTM:Activated by cleavage after Arg-444. The uncleaved zymogen is inactive towards synthetic substrates, but has sufficient activity to effect autocatalytic cleavage., PTM:The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains., similarity:Belongs to the peptidase S1 family., similarity:Contains 1 EGF-like domain., similarity:Contains 1 peptidase S1 domain., similarity:Contains 2 CUB domains., similarity:Contains 2 Sushi (CCP/SCR) domains., subunit:Homodimer; disulfide-linked. Binds MBL2. Isoform 2 binds to MASP1. Binds SERPING1., tissue specificity:Plasma.