CFP Human, Native

Properdin is a positive regulator of the alternative complement pathway. It binds to microbial surfaces and apoptotic cells, stabilizing the C3- and C5-convertase enzyme complexes. This stabilization is critical for the amplification of the complement response, which enhances the immune system’s ability to clear pathogens and damaged cells .

The gene encoding Complement Factor Properdin is known as CFP. It is located on the X chromosome and is responsible for producing the properdin protein. Mutations in the CFP gene can lead to properdin deficiency, which increases susceptibility to infections, particularly meningococcal infections .

Properdin plays a significant role in various diseases associated with complement dysregulation. For instance, in conditions like paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), properdin’s role in stabilizing the complement pathway can exacerbate the disease. Inhibiting properdin has been shown to prevent hemolysis in these conditions, making it a potential therapeutic target .

Recent studies have highlighted the importance of properdin in the pathogenesis of diseases involving complement dysregulation. Researchers have developed monoclonal antibodies targeting properdin, which have shown promise in preventing hemolysis in vitro models of PNH and aHUS . These findings suggest that properdin inhibitors could be a valuable addition to the therapeutic arsenal for treating complement-mediated diseases.