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C2

Component C2 is a serum glycoprotein that functions as part of the classical pathway of the complement system. Activated C1 cleaves C2 into C2a and C2b. The serine proteinase C2a then combines with complement factor 4b to create the C3 or C5 convertase. Deficiency of C2 has been reported to associated with certain autoimmune diseases and SNPs in this gene have been associated with altered susceptibility to age-related macular degeneration. This gene localizes within the class III region of the MHC on the short arm of chromosome 6. Alternative splicing results in multiple transcript variants encoding distinct isoforms. Additional transcript variants have been described in publications but their full-length sequence has not been determined.
Protein class

Disease related genes, Enzymes, Human disease related genes, Plasma proteins, Potential drug targets

Predicted location

Intracellular, Secreted (different isoforms)

Single cell type specificity

Group enriched (Hepatocytes, Hofbauer cells, Alveolar cells type 2, Kupffer cells)

Immune cell specificity

Group enriched (non-classical monocyte, intermediate monocyte)

Cell line specificity

Cell line enriched (Karpas-707)

Interaction

C2a interacts with Schistosoma haematobium TOR (via N-terminal extracellular domain). This results in inhibition of the classical and lectin pathway of complement activation, probably due to interference with binding of C2a to C4b such that C3 convertase cannot be formed. This infers resistance to complement-mediated cell lysis, allowing parasite survival and infection.

Molecular function

Hydrolase, Protease, Serine protease

More Types Infomation

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For Research Use Only. Not For Clinical Use.

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