MASP2base: MASP2 deficiency

Version 1.0 last updated 18 June, 2007 contains 1 public entries


Curated:	Mauno Vihinen
Maintained by:	Jouni Väliaho
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Short description of the MASP2base :

Mannan-binding lectin associated serine protease (MASP-2) is homologous to MASP-1 and the two C1q-associated serine proteases C1r and C1s. When mannan-binding lectin binds to carbohydrate structures on microorganisms, the manan-binding lectin pathway of the complement system is activated. By autoactivating MASP2 cleaves complement factors C4 and C2, generating the C3 convertase C4bC2b. Activation of C3 initiates the alternative pathway and the formation of the membrane-attack complex. Phagocytosis and inflammatory reactions are facilitated by complement fragments deposited on microorganisms. The MASP-2 deficiency is associated with susceptibility to infections and with the development of immunologic disease.

Other resourses:
Immunodeficiency Resource (IDR)
Immunodeficiency Mutation Databases (MUTbase)
IDdiagnostics

IDbases are linked to University of California Santa Cruz (UCSC) genome browser: MASP2base