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Details on Person UniProt:O00187-1 MASP2
| Class:Id | ReferenceIsoform:59007 |
|---|---|
| _chainChangeLog | signal peptide:1-15 added on Fri February 6 2015;chain:16-686 added on Fri February 6 2015;chain:16-444 added on Fri February 6 2015;chain:445-686 added on Fri February 6 2015 |
| _displayName | UniProt:O00187-1 MASP2 |
| _timestamp | 2026-02-20 22:01:10 |
| chain | signal peptide:1-15 chain:16-686 chain:16-444 chain:445-686 |
| checksum | ED952085FA115E21 |
| comment | FUNCTION Precursor of a serum protease that activates the lectin pathway of the complement system, a cascade of proteins that leads to phagocytosis and breakdown of pathogens and signaling that strengthens the adaptive immune system (PubMed:11527969, PubMed:22691502). The lectin complement system is activated following association of lectins, such as MBL2, FCN1, FCN2 or FCN3, to carbohydrates on the pathogen surface (PubMed:22691502, PubMed:22966085). MASP2 is cleaved and activated by MASP1 in response to lectin-binding to pathogen carbohydrates (PubMed:10946292, PubMed:22949645, PubMed:22966085, PubMed:9087411). Can activate prothrombin to thrombin (PubMed:39924859).FUNCTION Serine protease component of the lectin complement pathway, which catalyzes cleavage and activation of C2 and C4, the next components of the complement pathway.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).ACTIVITY REGULATION The protease activity is specifically inhibited by SGMI-2 small protein inhibitor.ACTIVITY REGULATION (Microbial infection) Serine protease activity is inhibited by E.Coli ecotin (eco).SUBUNIT Homodimer; disulfide-linked (PubMed:15117939, PubMed:15364579). Interacts with MBL2 and FCN2; requires calcium ions (PubMed:15117939). Interacts with SERPING1 (PubMed:10946292). Interacts with guianensin, an anticoagulant and anti-complement protein from Simulium guianense saliva; the interaction results in the inhibition of MASP2 enzymatic activity (PubMed:39924859).INTERACTION Recruited to the surface of pathogens by the mannose-binding proteins, such as MBL2.ALTERNATIVE PRODUCTS Plasma.PTM Activated by cleavage after Arg-444 by MASP1. 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.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the peptidase S1 family.ONLINE INFORMATION MASP2 mutation db |
| description | recommendedName: Mannan-binding lectin serine protease 2 ecNumber evidence="9 17 18 19 23"3.4.21.104 alternativeName: MBL-associated serine protease 2 alternativeName: fullName evidence="29"Mannose-binding protein-associated serine protease 2 shortName evidence="28 29"MASP-2 component recommendedName: Mannan-binding lectin serine protease 2 A chain /component component recommendedName: Mannan-binding lectin serine protease 2 B chain /component |
| geneName | MASP2 |
| identifier | O00187 |
| isoformParent | |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Alternative splicing Autocatalytic cleavage Calcium Complement pathway Direct protein sequencing Disease variant Disulfide bond EGF-like domain Hydrolase Hydroxylation Immunity Innate immunity Metal-binding Protease Proteomics identification Reference proteome Repeat Secreted Serine protease Signal Sushi |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9983091] Weiser, Joel, 2026-02-20 |
| name | MASP2 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8996090] ENSEMBL:ENSG00000009724 MASP2 [Homo sapiens] |
| secondaryIdentifier | MASP2_HUMAN A8K458 A8MWJ2 O75754 Q5TEQ5 Q5TER0 Q96QG4 Q9BZH0 Q9H498 Q9H499 Q9UBP3 Q9UC48 Q9ULC7 Q9UMV3 Q9Y270 |
| sequenceLength | 686 |
| species | [Species:48887] Homo sapiens |
| variantIdentifier | O00187-1 |
| (referenceEntity) | [EntityWithAccessionedSequence:166689] MASP2-1 [extracellular region] [Homo sapiens] [EntityWithAccessionedSequence:166703] MASP2-1(445-686) [extracellular region] [Homo sapiens] [EntityWithAccessionedSequence:166705] MASP2-1(16-444) [extracellular region] [Homo sapiens] |
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No pathways have been reviewed or authored by UniProt:O00187-1 MASP2 (59007)
