Complement Component C8
C8 is a glycoprotein composed of three nonidentical subunits (C8α, C8β, and C8γ) that are encoded by three separate genes. C8α and C8β are members of the membrane attack complex (MAC) family of structurally related proteins. In contrast, C8γ is unrelated and is a member of the lipocalin family of widely distributed proteins that bind and transport small lipophilic ligands.
C8 is a component of the MAC that forms pores in the membranes of cells of invading organisms. It plays a key role in the formation of the MAC. It initiates membrane penetration and coordinates the formation of MAC pores. MAC forms when complement factors C5 is cleaved into C5a and C5b, and C5b binds complement factors C6, complement factors C7, C8, and complement factors C9 molecules in sequence, which results in transmembrane pores and eventually cell lysis. After binding to C8, the C5b-7 complex itself is transiently bound to the membrane surface and has no function, and is given the ability to cause membrane destruction and C9 polymerization to form the C5b-9 complex (also known as MAC).
Fig. 1 3D structure of C8 complex.1
Complement Component C8 Deficiency
Complement component C8 deficiency is a disorder that causes dysfunction of the immune system, leading to some form of immune deficiency. Immune deficiency refers to the inability of the immune system to effectively protect the body from foreign invaders such as bacteria. People with C8 deficiency have a significantly increased risk of recurrent infections with bacteria, especially bacteria known as Neisseria meningitidis. Although meningitis may be life-threatening, people with C8 deficiency are less likely to die from the infection than the general population who is infected with it.
Complement component 8 deficiency is classified into type I and type II according to its genetic cause. These two types have the same signs and symptoms. In addition, complement component 8 deficiency is a very rare disease, however, its prevalence is unknown. Type I occurs in several populations, especially those with Hispanic, Japanese, or African Caribbean heritage, while type II occurs mainly in people of Northern European descent. The severity of complement component 8 deficiency varies widely. Although some people have the disease, others do not have any health problems associated with the disease.
Creative Biolabs offers a range of complement component C8 related services and products, including:
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Antibodies (C8A Antibodies, C8B Antibodies and C8G Antibodies)
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Proteins (C8A Proteins, C8B Proteins and C8G Proteins)
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Array Kits (C8A Array Kits, C8B Array Kits and C8G Array Kits)
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Peptides (C8B Peptides and C8G Peptides)
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Lysates (C8A Lysates, C8B Lysates and C8G Lysates)
If you have any questions about our products, please feel free to contact us.
Reference
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From Wikipedia: By SchauderCM - Own work, CC BY-SA 3.0 https://commons.wikimedia.org/wiki/File:3OJY.png
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