Entrez Summary: This gene encodes a plasma glycoprotein that positively regulates the alternative complement pathway of the innate immune system. This protein binds to many microbial surfaces and apoptotic cells and stabilizes the C3- and C5-convertase enzyme complexes in a feedback loop that ultimately leads to formation of the membrane attack complex and lysis of the target cell. Mutations in this gene result in two forms of properdin deficiency, which results in high susceptibility to meningococcal infections. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Feb 2009].
UniProt Summary: A positive regulator of the alternate pathway of complement. It binds to and stabilizes the C3- and C5-convertase enzyme complexes. {ECO:0000269|PubMed:20382442}.
Pfam DomainsGO Terms
Pfam Domains
TSP 1
GO Terms
complement activation, alternative pathway
tertiary granule lumen
specific granule lumen
regulation of complement activation
regulation of humoral immune response
complement activation
endoplasmic reticulum lumen
defense response to bacterium
humoral immune response
collagen-containing extracellular matrix
regulation of immune effector process
neutrophil degranulation
neutrophil activation involved in immune response
neutrophil mediated immunity
neutrophil activation
granulocyte activation
leukocyte degranulation
myeloid leukocyte mediated immunity
myeloid cell activation involved in immune response
myeloid leukocyte activation
leukocyte activation involved in immune response
cell activation involved in immune response
activation of immune response
response to bacterium
regulated exocytosis
innate immune response
leukocyte mediated immunity
exocytosis
positive regulation of immune response
leukocyte activation
defense response to other organism
secretion by cell
export from cell
cell activation
immune effector process
secretion
regulation of immune response
positive regulation of immune system process
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)