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Properdin

Properdin plasma glycoprotein is encoded by the CFP (complement factor properdin) gene.

Properdin is plasma glycoprotein that activates the complement system of the innate immune system. 

Properdin is a protein that plays a crucial role in the immune system’s defense against infections. 

It is part of the complement system, which is a complex network of proteins that work together to enhance the ability of antibodies and immune cells to eliminate pathogens.

The main function of properdin is to stabilize and activate the alternative pathway of the complement system. 

The alternative pathway of the complement system is an important component of the immune response, especially in the early stages of an infection. 

Properdin binds to certain surfaces of pathogens, such as bacteria, and helps in the recruitment and activation of other complement proteins.

It also helps prevent an excessive immune response by promoting the breakdown of complement proteins, ensuring a balanced and controlled immune reaction.

The alternative pathway is not dependent on antibodies. 

Deficiencies in properdin can lead to an increased susceptibility to certain bacterial infections, particularly those caused by encapsulated bacteria: meningitis, sepsis, and pneumonia.

This protein binds to bacterial cell walls and dying human cells to stabilize the C3 and C5-convertase enzyme complexes.

It form an attack complex that lead to the lysis of the cell.

Properdin gamma globulin protein is composed of multiple identical protein subunits with a separate ligand-binding site, and occurs in dimers, trimers and tetramers in the fixed ratio 22:52:28.

Properdin participates in specific immune responses, and plays a part in tissue inflammation as well as the engulfing of pathogens by phagocytes. 

Properdin helps to neutralize some viruses.

The alternative branch of the complement system is activated by IgA immune complexes and bacterial endotoxins, polysaccharides, and cell walls.

Activation of the alternative complement pathway  results in producing anaphylatoxins, opsonins, chemotactic factors, and the membrane attack complex, all of which help fight pathogens.

Properdin deficiency is a rare X-linked disease in which properdin is deficient. 

Affected individuals are susceptible to fulminant meningococcal disease.

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