Complement System - Role in Disease

Role in Disease

It is thought that the complement system might play a role in many diseases with an immune component, such as Barraquer-Simons Syndrome, asthma, lupus erythematosus, glomerulonephritis, various forms of arthritis, autoimmune heart disease, multiple sclerosis, inflammatory bowel disease, and ischemia-reperfusion injuries. and rejection of transplanted organs.

The complement system is also becoming increasingly implicated in diseases of the central nervous system such as Alzheimer's disease and other neurodegenerative conditions such as spinal cord injuries.

Deficiencies of the terminal pathway predispose to both autoimmune disease and infections (particularly Neisseria meningitidis, due to the role that the membrane attack complex plays in attacking Gram-negative bacteria).

Mutations in the complement regulators factor H and membrane cofactor protein have been associated with atypical haemolytic uraemic syndrome. Moreover, a common single nucleotide polymorphism in factor H (Y402H) has been associated with the common eye disease age-related macular degeneration. Polymorphisms of complement component 3, complement factor B, and complement factor I, as well as deletion of complement factor H-related 3 and complement factor H-related 1 also affect a person's risk of developing age-related macular degeneration. Both of these disorders are currently thought to be due to aberrant complement activation on the surface of host cells.

Mutations in the C1 inhibitor gene can cause hereditary angioedema, an autoimmune condition resulting from reduced regulation of the complement pathway.

Mutations in the MAC components of complement, especially C8, are often implicated in recurrent Neisserial infection.

Diagnostic tools to measure complement activity include the total complement activity test.

Read more about this topic:  Complement System

Famous quotes containing the words role in, role and/or disease:

    Language makes it possible for a child to incorporate his parents’ verbal prohibitions, to make them part of himself....We don’t speak of a conscience yet in the child who is just acquiring language, but we can see very clearly how language plays an indispensable role in the formation of conscience. In fact, the moral achievement of man, the whole complex of factors that go into the organization of conscience is very largely based upon language.
    Selma H. Fraiberg (20th century)

    Where we come from in America no longer signifies—it’s where we go, and what we do when we get there, that tells us who we are.
    The irony of the role of women in my business, and in so many other places, too, was that while we began by demanding that we be allowed to mimic the ways of men, we wound up knowing we would have to change those ways. Not only because those ways were not like ours, but because they simply did not work.
    Anna Quindlen (b. 1952)

    There is a disease to which plays as well as men become liable with advancing years. In men it is called doting, in plays dating. The more topical the play the more it dates.
    George Bernard Shaw (1856–1950)