Mannose Associated Serine Protease 2 (MASP2)

Mannose-binding protein-associated serine protease 2; mannan-Binding Lectin Serine Peptidase 2; MBL-associated serine protease 2

SPN	V952
HGNC	6902
MGI	1330832
PubMed	15672593
RGD	620214
UniProt	O00187
Wiki	MASP2

MASP2 is a protein involved in the complement system. Mannose-binding lectin (MBL) is a serum component which participates in innate immunity by activating complement via a novel pathway. Human MBL forms complexes with two types of serine proteases termed MASP (MBL-associated serine protease). These two proteases, MASP1 and MASP2, are structurally similar to one another as well as to C1r and C1s. Together, MASP, C1r and C1s constitute a novel serine protease family. It is likely that human MASP1 is able to activate C3, while human MASP2 cleaves C4, although further functional studies are required to confirm this. Based on the analysis of MASP cDNA of vertebrates and ascidians, the MASP/C1r/C1s family can be classified into two groups.

Reference

"A second serine protease associated with mannan-binding lectin that activates complement."
Nature 386:506-510(1997) [PubMed] [Europe PMC] [Abstract]

"A truncated form of mannose-binding lectin-associated serine protease (MASP)-2 expressed by alternative polyadenylation is a component of the lectin complement pathway."
Int. Immunol. 11:859-863(1999) [PubMed] [Europe PMC] [Abstract]

"Two constituents of the initiation complex of the mannan-binding lectin activation pathway of complement are encoded by a single structural gene."
J. Immunol. 162:3481-3490(1999) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence and biological annotation of human chromosome 1."
Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]

"Towards a comprehensive database of proteins from the urine of patients with bladder cancer."
J. Urol. 155:2113-2119(1996) [PubMed] [Europe PMC] [Abstract]

"The human gene for mannan-binding lectin-associated serine protease-2 (MASP-2), the effector component of the lectin route of complement activation, is part of a tightly linked gene cluster on chromosome 1p36.2-3."
Genes Immun. 2:119-127(2001) [PubMed] [Europe PMC] [Abstract]

"Proteolytic activities of two types of mannose-binding lectin-associated serine protease."
J. Immunol. 165:2637-2642(2000) [PubMed] [Europe PMC] [Abstract]

"The X-ray structure of human mannan-binding lectin-associated protein 19 (MAp19) and its interaction site with mannan-binding lectin and L-ficolin."
J. Biol. Chem. 279:29391-29397(2004) [PubMed] [Europe PMC] [Abstract]

"The structure of MBL-associated serine protease-2 reveals that identical substrate specificities of C1s and MASP-2 are realized through different sets of enzyme-substrate interactions."
J. Mol. Biol. 342:1533-1546(2004) [PubMed] [Europe PMC] [Abstract]

"A true autoactivating enzyme. Structural insight into mannose-binding lectin-associated serine protease-2 activations."
J. Biol. Chem. 280:33435-33444(2005) [PubMed] [Europe PMC] [Abstract]

"Inherited deficiency of mannan-binding lectin-associated serine protease 2."
N. Engl. J. Med. 349:554-560(2003) [PubMed] [Europe PMC] [Abstract]

"Novel MASP2 variants detected among North African and Sub-Saharan individuals."
Tissue Antigens 66:131-135(2005) [PubMed] [Europe PMC] [Abstract]

"Deficiency of mannan-binding lectin associated serine protease-2 due to missense polymorphisms."
Genes Immun. 8:154-163(2007) [PubMed] [Europe PMC] [Abstract]