NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
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: EC 3.4.21.43; Protease; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 6p21.3
Cellular Component: extracellular region
Molecular Function: serine-type endopeptidase activity
Biological Process: complement activation; regulation of complement activation
Disease: Complement Component 2 Deficiency; Macular Degeneration, Age-related, 14
Research Articles on C2
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Product Notes
The Human C2 c2 (Catalog #AAA761810) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA761810 ELISA Kit recognizes Human C2. It is sometimes possible for the material contained within the vial of "Complement C2, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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