NP_002612.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
properdin
NCBI Official Synonym Full Names
complement factor properdin
NCBI Protein Information
properdin; complement factor P; properdin P factor, complement
UniProt Protein Name
Properdin
UniProt Synonym Protein Names
Complement factor P
UniProt Synonym Gene Names
UniProt Entry Name
PROP_HUMAN
NCBI Summary for CFP
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 Comments for CFP
CFP: A positive regulator of the alternate pathway of complement. It binds to and stabilizes the C3- and C5-convertase enzyme complexes. Defects in CFP are the cause of properdin deficiency (PFD). PFD results in higher susceptibility to bacterial infections; especially to meningococcal infections. Three phenotypes have been reported: complete deficiency (type I), incomplete deficiency (type II), and dysfunction of properdin (type III).
Protein type: Secreted, signal peptide; Secreted
Chromosomal Location of Human Ortholog: Xp11.4
Cellular Component: extracellular matrix; extracellular space; endoplasmic reticulum lumen; extracellular region
Biological Process: protein amino acid O-linked glycosylation; cellular protein metabolic process; defense response to bacterium; complement activation, alternative pathway; regulation of complement activation; innate immune response; immune response; post-translational protein modification; complement activation
Disease: Properdin Deficiency, X-linked
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Products associated with anti-CFP antibody
Pathways associated with anti-CFP antibody
Diseases associated with anti-CFP antibody
Organs/Tissues associated with anti-CFP antibody
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