Properdin deficiency
Encyclopedia
Properdin deficiency is a rare X-linked disease in which properdin
, an important complement factor
, is deficient. Affected individuals are susceptible to fulminant
meningococcal disease
.
Properdin
Properdin or factor P is a globulin protein found in the blood serum of more complex animals. In the complement system, an innate-immunity series of proenzymes dissolved in the circulation, it is also called "Factor P".-Function:...
, an important complement factor
Complement system
The complement system helps or “complements” the ability of antibodies and phagocytic cells to clear pathogens from an organism. It is part of the immune system called the innate immune system that is not adaptable and does not change over the course of an individual's lifetime...
, is deficient. Affected individuals are susceptible to fulminant
Fulminant
Fulminant is any event or process that occurs suddenly and quickly, and is intense and severe to the point of lethality, i.e., it has an explosive character. The word comes from Latin fulmināre, to strike with lightning...
meningococcal disease
Meningococcemia
Meningococcal disease describes infections caused by the bacterium Neisseria meningitidis . It carries a high mortality rate if untreated. While best known as a cause of meningitis, widespread blood infection is more damaging and dangerous...
.