CFP (Properdin) (CFP) ELISA Kit for Rat

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]

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; Secreted, signal peptide

Chromosomal Location of Human Ortholog: Xp11.4

Cellular Component: endoplasmic reticulum lumen; extracellular matrix; extracellular region; extracellular space

Biological Process: complement activation; complement activation, alternative pathway; defense response to bacterium; immune response; regulation of complement activation

Disease: Properdin Deficiency, X-linked