MASP2 Antibody – Rabbit Polyclonal

The Ra-reactive factor (RARF) is a complement-dependent bactericidal factor that binds to the Ra and R2 polysaccharides expressed by certain enterobacteria. Alternate splicing of this gene results in two transcript variants encoding two RARF components that are involved in the mannan-binding lectin pathway of complement activation. The longer isoform is cleaved into two chains which form a heterodimer linked by a disulfide bond. The encoded proteins are members of the trypsin family of peptidases. [provided by RefSeq, Jul 2008]

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

Chromosomal Location of Human Ortholog: 1p36.3-p36.2

Cellular Component: extracellular region

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

Biological Process: innate immune response; proteolysis; complement activation, lectin pathway; complement activation, classical pathway; complement activation

Disease: Masp2 Deficiency