ID:MASP2_HUMAN DESCRIPTION: RecName: Full=Mannan-binding lectin serine protease 2; EC=3.4.21.104; AltName: Full=MBL-associated serine protease 2; AltName: Full=Mannose-binding protein-associated serine protease 2; Short=MASP-2; Contains: RecName: Full=Mannan-binding lectin serine protease 2 A chain; Contains: RecName: Full=Mannan-binding lectin serine protease 2 B chain; Flags: Precursor; 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. 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). SUBUNIT: Homodimer; disulfide-linked. Binds MBL2. Isoform 2 binds to MASP1. Binds SERPING1. Dimerization and MBL2 binding requires calcium ions. SUBCELLULAR LOCATION: Secreted. TISSUE SPECIFICITY: Plasma. PTM: The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains (By similarity). PTM: Activated by cleavage after Arg-444. The uncleaved zymogen is inactive towards synthetic substrates, but has sufficient activity to effect autocatalytic cleavage. DISEASE: Defects in MASP2 are the cause of MASP2 deficiency (MASPD) [MIM:613791]. MASPD is a disorder that results in autoimmune manifestations, recurrent severe infections, and chronic inflammatory disease. SIMILARITY: Belongs to the peptidase S1 family. SIMILARITY: Contains 2 CUB domains. SIMILARITY: Contains 1 EGF-like domain. SIMILARITY: Contains 1 peptidase S1 domain. SIMILARITY: Contains 2 Sushi (CCP/SCR) domains. WEB RESOURCE: Name=MASP2base; Note=MASP2 mutation db; URL="http://bioinf.uta.fi/MASP2base/";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on O00187
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
JD154552 - Sequence 135576 from Patent EP1572962. Y09926 - Homo sapiens mRNA for MASP-2 protein. X98400 - H.sapiens mRNA for mannan-binding lectin-associated serine protease-2. JD490652 - Sequence 471676 from Patent EP1572962. JD381855 - Sequence 362879 from Patent EP1572962. JD093995 - Sequence 75019 from Patent EP1572962. JD424268 - Sequence 405292 from Patent EP1572962. AK290823 - Homo sapiens cDNA FLJ77090 complete cds, highly similar to Homo sapiens mannan-binding lectin serine peptidase 2 (MASP2), transcript variant 1, mRNA. BC156886 - Synthetic construct Homo sapiens clone IMAGE:100062765, MGC:190607 mannan-binding lectin serine peptidase 2 (MASP2) mRNA, encodes complete protein. BC156086 - Synthetic construct Homo sapiens clone IMAGE:100062346, MGC:190474 mannan-binding lectin serine peptidase 2 (MASP2) mRNA, encodes complete protein. Y18282 - Homo sapiens mRNA for mannose binding lectin-associated serine protease-2, alternatively spliced transcript (clone phl-7). Y18283 - Homo sapiens mRNA for mannose binding lectin-associated serine protease-2, alternatively spliced transcript (clone phl-6). BC052299 - Homo sapiens cDNA clone IMAGE:6285857, partial cds. BC067359 - Homo sapiens mannan-binding lectin serine peptidase 2, mRNA (cDNA clone IMAGE:6299026), with apparent retained intron. BC080556 - Homo sapiens cDNA clone IMAGE:6269223. Y18281 - Homo sapiens mRNA for mannose binding lectin-associated serine protease-2, alternatively spliced transcript (clone phl-5). Y18284 - Homo sapiens mRNA for mannose binding lectin-associated serine protease-2, incompletely spliced primary transcript, clone phl-8. AB008047 - Homo sapiens sMAP mRNA for small MBL-associated protein, complete cds.
Biochemical and Signaling Pathways
BioCarta from NCI Cancer Genome Anatomy Project h_compPathway - Complement Pathway h_lectinPathway - Lectin Induced Complement Pathway
Reactome (by CSHL, EBI, and GO)
Protein O00187 (Reactome details) participates in the following event(s):
R-HSA-166721 MBL binds to repetitive carbohydrate structures on the surfaces of viruses, bacteria, fungi, and protozoa R-HSA-2855054 Ficolin-2 binds to molecular patterns on the target cell surface R-HSA-2855125 Ficolin-1 binds to molecular patterns on the target cell surface R-HSA-2855077 Ficolin-3 binds to molecular patterns on the target cell surface R-HSA-8852509 CL-LK binds carbohydrates on target cell surface R-HSA-166726 Activation of MASPs R-HSA-166753 Conversion of C4 into C4a and C4b R-HSA-166792 Conversion of C2 into C2a and C2b R-HSA-166662 Lectin pathway of complement activation R-HSA-2855086 Ficolins bind to repetitive carbohydrate structures on the target cell surface R-HSA-166786 Creation of C4 and C2 activators R-HSA-166663 Initial triggering of complement R-HSA-166658 Complement cascade R-HSA-168249 Innate Immune System R-HSA-168256 Immune System
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
