Entrez Summary: This gene encodes a serine protease that functions as a component of the lectin pathway of complement activation. The complement pathway plays an essential role in the innate and adaptive immune response. The encoded protein is synthesized as a zymogen and is activated when it complexes with the pathogen recognition molecules of lectin pathway, the mannose-binding lectin and the ficolins. This protein is not directly involved in complement activation but may play a role as an amplifier of complement activation by cleaving complement C2 or by activating another complement serine protease, MASP-2. The encoded protein is also able to cleave fibrinogen and factor XIII and may may be involved in coagulation. A splice variant of this gene which lacks the serine protease domain functions as an inhibitor of the complement pathway. Alternate splicing results in multiple transcript variants.[provided by RefSeq, Apr 2010].
UniProt Summary: N/A
Pfam DomainsGO Terms
Pfam Domains
CUB
Trypsin
Sushi
EGF CA
GO Terms
complement activation, lectin pathway
calcium-dependent protein binding
peptidase activity
serine-type endopeptidase activity
complement activation
receptor-mediated endocytosis
humoral immune response
endocytosis
activation of immune response
import into cell
calcium ion binding
innate immune response
positive regulation of immune response
protein homodimerization activity
defense response to other organism
immune effector process
regulation of immune response
positive regulation of immune system process
proteolysis
response to other organism
response to external biotic stimulus
response to biotic stimulus
defense response
extracellular space
regulation of immune system process
immune response
extracellular region
vesicle-mediated transport
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)
Compound Hit
No hits were found.
Most Correlated Genes in Chemogenomics
No correlation found to any other genes in chemogenomics.
Tissues where Essential in the Avana Dataset (DepMap 20Q1)
Global Fraction of Cell Lines Where Essential: 0/739