NP_006601
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
mannan-binding lectin serine protease 2 isoform 2
NCBI Official Synonym Full Names
mannan binding lectin serine peptidase 2
NCBI Protein Information
mannan-binding lectin serine protease 2
UniProt Protein Name
Mannan-binding lectin serine protease 2
UniProt Synonym Protein Names
MBL-associated serine protease 2; Mannose-binding protein-associated serine protease 2; MASP-2
UniProt Synonym Gene Names
UniProt Entry Name
MASP2_HUMAN
NCBI Summary for MASP2
This gene encodes a member of the peptidase S1 family of serine proteases. The encoded preproprotein is proteolytically processed to generate A and B chains that heterodimerize to form the mature protease. This protease cleaves complement components C2 and C4 in order to generate C3 convertase in the lectin pathway of the complement system. The encoded protease also plays a role in the coagulation cascade through cleavage of prothrombin to form thrombin. Myocardial infarction and acute stroke patients exhibit reduced serum concentrations of the encoded protein. Alternative splicing results in multiple transcript variants, at least one of which encodes an isoform that is proteolytically processed. [provided by RefSeq, Feb 2016]
UniProt Comments for MASP2
MASP2: 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. Defects in MASP2 are the cause of MASP2 deficiency (MASPD). MASPD is a disorder that results in autoimmune manifestations, recurrent severe infections, and chronic inflammatory disease. Belongs to the peptidase S1 family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 3.4.21.104; Protease; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 1p36.3-p36.2
Cellular Component: extracellular region; extracellular space
Molecular Function: calcium-dependent protein binding; complement component C4b binding; peptidase activity; protein binding; serine-type endopeptidase activity
Biological Process: complement activation; complement activation, lectin pathway
Disease: Masp2 Deficiency
Research Articles on MASP2
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Products associated with anti-MASP2 antibody
Pathways associated with anti-MASP2 antibody
Diseases associated with anti-MASP2 antibody
Organs/Tissues associated with anti-MASP2 antibody
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