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Complement factor I, also known as C3b/C4b inactivator, is a protein that in humans is encoded by the CFI gene. Complement factor I (factor I) is a protein of the complement system , first isolated in 1966 in guinea pig serum , [ 5 ] that regulates complement activation by cleaving cell-bound or fluid phase C3b and C4b. [ 6 ]
First, the proteolytic component of the convertase, Bb, is removed by complement regulatory proteins having decay-accelerating factor (DAF) activity. Next, C3b is broken down progressively to first iC3b, then C3c + C3dg, and then finally C3d. Factor I is the protease cleaves C3b but requires a cofactor (e.g Factor H, CR1, MCP or C4BP) for activity.
Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins. [4] Because of redundancies in the immune system, many complement disorders are never diagnosed. Some studies estimate that less than 10% are identified. [5]
The typical tests for complement deficiencies, such as the measurement of C3 and C4, do not detect low levels of the absence of properdin. [2] These pathways are typically unaltered by any of the three types of properdin deficiencies, but even when they are affected, it is typically within normal levels and is not cause for concern. [ 2 ]
The complement system is part of the innate as well as the adaptive immune system; it is a group of circulating proteins that can bind pathogens and form a membrane attack complex. Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to autoimmune conditions.
Complement is responsible for immune inflammatory response in adipose tissues which has been implicated in the development of obesity. [8] Obesity in turn results in an abnormally high level of complement activation via production of the C1 component of the classical pathway, which can lead to tissue inflammation and eventually insulin ...
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The classical and alternative complement pathways. Alternative pathway. (Some labels are in Polish.) The alternative pathway is a type of cascade reaction of the complement system and is a component of the innate immune system, a natural defense against infections.