MASP2
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.
Involvement in disease
MASP2 deficiency
MASPD
A disorder that results in autoimmune manifestations, recurrent severe infections, and chronic inflammatory disease.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.
Activated by cleavage after Arg-444. The uncleaved zymogen is inactive towards synthetic substrates, but has sufficient activity to effect autocatalytic cleavage.
Sequence Similarities
Belongs to the peptidase S1 family.
Tissue Specificity
Plasma.
Cellular localization
- Secreted
Alternative names
Mannan-binding lectin serine protease 2, MBL-associated serine protease 2, Mannose-binding protein-associated serine protease 2, MASP-2, MASP2