功能: 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.,
功能: 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.,