Complement component 5 is a
protein
Proteins are large biomolecules and macromolecules that comprise one or more long chains of amino acid residues. Proteins perform a vast array of functions within organisms, including catalysing metabolic reactions, DNA replication, respo ...
that in humans is encoded by the ''C5''
gene
In biology, the word gene (from , ; "...Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a ba ...
.
Complement component 5 is involved in the
complement system
The complement system, also known as complement cascade, is a part of the immune system that enhances (complements) the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promote inflammation, and at ...
. It is cleaved into
C5a and C5b:
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C5a plays an important role in
chemotaxis
Chemotaxis (from '' chemo-'' + ''taxis'') is the movement of an organism or entity in response to a chemical stimulus. Somatic cells, bacteria, and other single-cell or multicellular organisms direct their movements according to certain chemica ...
.
* C5b forms the first part of the
complement membrane attack complex.
Deficiency is thought to cause
Leiner's disease
Leiner's disease is a systemic disease, a skin disorder and extends to erythroderma, typically diagnosed in early infancy. Leiner's disease is characterized by a long-lasting seborrhea dermatitis associated with increased likelihood to infection. ...
.
Function
Complement component 5 is the fifth component of complement, which plays an important role in inflammatory and cell killing processes. This protein is composed of alpha and beta polypeptide chains that are linked by a disulfide bridge. An activation peptide, C5a, which is an
anaphylatoxin that possesses potent
spasmogenic and
chemotactic activity, is derived from the alpha polypeptide via cleavage with a
C5-convertase
C5 convertase is an enzyme belonging to a family of serine proteases that play key role in the innate immunity. It participates in the complement system ending with cell death.
There are four different C5 convertases able to specifically con ...
. The C5b macromolecular cleavage product can form a complex with the
C6 complement component, and this complex is the basis for formation of the membrane attack complex, which includes additional complement components.
Clinical significance
Mutations in this gene cause complement component 5 deficiency, a disease where patients show a propensity for severe recurrent infections. Defects in this gene have also been linked to a susceptibility to
liver fibrosis
Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, and end-stage liver disease, is the impaired liver function caused by the formation of scar tissue known as fibrosis due to damage caused by liver disease. Damage causes tissue repai ...
and to
rheumatoid arthritis
Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are involv ...
.
Therapeutic applications
The drug
eculizumab
Eculizumab, sold under the brand name Soliris among others, is a medication used to treat paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), generalized myasthenia gravis, and neuromyelitis optica. In people ...
(trade name Soliris) prevents cleavage of C5 into C5a and C5b.
Complement system pathway
References
Further reading
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External links
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Complement system
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