Complement C2; C3/C5 convertase; Complement C2b fragment; Complement C2a fragment; C2

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This C2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 147-176 amino acids from the N-terminal region of human C2.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.26 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-C2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Immunology

Western Blot (WB), ELISA (EIA)

WB~~1:1000

C2 Antibody (N-term) western blot analysis in CHO cell line lysates (35ug/lane).This demonstrates the C2 antibody detected the C2 protein (arrow).

83268

complement component 2

complement C2

Complement C2

CO2_HUMAN

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.[provided by RefSeq, Mar 2009]

C2: Component C2 which is part of the classical pathway of the complement system is cleaved by activated factor C1 into two fragments: C2b and C2a. C2a, a serine protease, then combines with complement factor 4b to generate the C3 or C5 convertase. Defects in C2 are the cause of complement component 2 deficiency (C2D). A deficiency of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus erythematosus. Skin and joint manifestations are common and renal disease is relatively rare. Patients with complement component 2 deficiency are also reported to have recurrent or invasive infections. Belongs to the peptidase S1 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted; Secreted, signal peptide; Protease; EC 3.4.21.43

Chromosomal Location of Human Ortholog: 6p21.3

Cellular Component: extracellular space; extracellular region

Molecular Function: metal ion binding; serine-type endopeptidase activity

Biological Process: regulation of complement activation; innate immune response; proteolysis; complement activation, classical pathway; response to nutrient; complement activation

Disease: Macular Degeneration, Age-related, 14; Complement Component 2 Deficiency

Hu, M., et al. Pharmacogenet. Genomics 20(10):634-637(2010)
Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Liu, X., et al. Retina (Philadelphia, Pa.) 30(8):1177-1184(2010)
Han, S., et al. Hum. Immunol. 71(7):727-730(2010)
Ishii, Y., et al. J. Immunol. 151(1):170-174(1993)

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