产品名称
CO2 Rabbit Polyclonal Antibody
蛋白名称
Complement C2 (EC 3.4.21.43) (C3/C5 convertase) [Cleaved into: Complement C2b fragment; Complement C2a fragment]
存储缓冲液
Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.
Human Swissprot No.
P06681
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P06681/entry
Mouse Swissprot No.
P21180
Mouse Swissprot Link
http://www.uniprot.org/uniprot/P21180
免疫原
Synthesized peptide derived from part region of human protein
特异性
CO2 Polyclonal Antibody detects endogenous levels of protein.
稀释度
WB 1:500-2000 ELISA 1:5000-20000
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
Adipose tissue,Brain,Colon,Liver,Plasma,Small intestine,
信号通路
Complement and coagulation cascades;Systemic lupus erythematosus;
功能
catalytic activity:Selective cleavage of Arg-|-Ser bond in complement component C3 alpha-chain to form C3a and C3b, and Arg-|-Xaa bond in complement component C5 alpha-chain to form C5a and C5b.,disease:Defects in C2 are the cause of C2 deficiency (CD2D) [MIM:217000]. CD2D is an autosomal recessive disease. Deficient individuals have an increased incidence of SLE and SLE-like syndromes, glomerulonephritis, vasculitis and pyogenic infections. Type I C2D is characterized by complete loss of the protein while type II C2D is characterized by a selective block in C2 secretion.,function: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.,miscellaneous:C2 is a major histocompatibility complex class-III protein.,online information:C2 mutation db,polymorphism:The variant Asp-318 is associated with a reduced risk of age-related macular degeneration (ARMD) [MIM:603075]. ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world.,similarity:Belongs to the peptidase S1 family.,similarity:Contains 1 peptidase S1 domain.,similarity:Contains 1 VWFA domain.,similarity:Contains 3 Sushi (CCP/SCR) domains.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.