UniProt:O00187-1 MASP2

chain
  • signal peptide:1-15
  • chain:16-686
  • chain:16-444
  • chain:445-686
checksum ED952085FA115E21
comment
  • 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.INTERACTION Plasma.PTM The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.PTM Activated by cleavage after Arg-444. The uncleaved zymogen is inactive towards synthetic substrates, but has sufficient activity to effect autocatalytic cleavage.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
crossReference
databaseName UniProt
dbId 59007
description
  • recommendedName: Mannan-binding lectin serine protease 2 ecNumber: 3.4.21.104 alternativeName: MBL-associated serine protease 2 alternativeName: Mannose-binding protein-associated serine protease 2 shortName: MASP-2 component recommendedName: Mannan-binding lectin serine protease 2 A chain /component component recommendedName: Mannan-binding lectin serine protease 2 B chain /component
displayName UniProt:O00187-1 MASP2
geneName
  • MASP2
identifier O00187
isSequenceChanged false
isoformParent
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
  • Reference proteome
  • Repeat
  • Secreted
  • Serine protease
  • Signal
  • Sushi
modified [InstanceEdit:12187927] Wright, Adam, 2024-03-12
name
  • MASP2
otherIdentifier
  • 10747
  • 11735645_a_at
  • 11740562_at
  • 16681595
  • 207041_PM_at
  • 207041_at
  • 210798_3p_x_at
  • 210798_PM_x_at
  • 210798_x_at
  • 2396419
  • 2396420
  • 2396421
  • 2396422
  • 2396423
  • 2396427
  • 2396430
  • 2396431
  • 2396432
  • 2396434
  • 2396438
  • 2396440
  • 2396442
  • 2396444
  • 2396445
  • 2396447
  • 37514_s_at
  • 37515_at
  • 7912361
  • A_23_P301971
  • A_33_P3247571
  • A_33_P3307990
  • GE81659
  • GO:0001855
  • GO:0001867
  • GO:0002376
  • GO:0003824
  • GO:0004252
  • GO:0005509
  • GO:0005515
  • GO:0005576
  • GO:0005615
  • GO:0006508
  • GO:0006956
  • GO:0006958
  • GO:0008233
  • GO:0008236
  • GO:0016787
  • GO:0042802
  • GO:0043226
  • GO:0045087
  • GO:0046872
  • GO:0048306
  • GO:0070062
  • GO:0098542
  • GO:0110165
  • GO:0140096
  • HMNXSV003048003
  • ILMN_1709408
  • ILMN_1727723
  • PH_hs_0006621
  • TC01002194.hg
  • TC01005262.hg
  • g5002493_3p_a_at
  • g5002493_3p_x_at
  • g5729914_3p_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceIsoform
secondaryIdentifier
  • MASP2_HUMAN
  • A8K458
  • A8MWJ2
  • O75754
  • Q5TEQ5
  • Q5TER0
  • Q96QG4
  • Q9BZH0
  • Q9H498
  • Q9H499
  • Q9UBP3
  • Q9UC48
  • Q9ULC7
  • Q9UMV3
  • Q9Y270
sequenceLength 686
species [Species:48887] Homo sapiens
url https://purl.uniprot.org/uniprot/O00187-1
variantIdentifier O00187-1
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