蛋白名称
Complement C1q subcomponent subunit C
免疫原
The antiserum was produced against synthesized peptide derived from human C1QC. AA range:81-130
特异性
C1q-C Polyclonal Antibody detects endogenous levels of C1q-C protein.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
来源
Polyclonal, Rabbit,IgG
稀释比例
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/40000.. IF 1:50-200
纯化工艺
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
其他名称
C1QC; C1QG; Complement C1q subcomponent subunit C
背景
This gene encodes a major constituent of the human complement subcomponent C1q. C1q associates with C1r and C1s in order to yield the first component of the serum complement system. A deficiency in C1q has been associated with lupus erythematosus and glomerulonephritis. C1q is composed of 18 polypeptide chains: six A-chains, six B-chains, and six C-chains. Each chain contains a collagen-like region located near the N-terminus, and a C-terminal globular region. The A-, B-, and C-chains are arranged in the order A-C-B on chromosome 1. This gene encodes the C-chain polypeptide of human complement subcomponent C1q. Alternatively spliced transcript variants that encode the same protein have been found for this gene. [provided by RefSeq, Jul 2008],
功能
disease:Defects in C1QC are a cause of C1q deficiency [MIM:120575]. It is a rare genetic disorder which is associated with recurrent infections and a high prevalence of lupus erythematosus-like symptoms. It is characterized by a loss of activation of the complement classical pathway.,function:C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen-like regions of C1q interact with the Ca(2+)-dependent C1r(2)C1s(2) proenzyme complex, and efficient activation of C1 takes place on interaction of the globular heads of C1q with the Fc regions of IgG or IgM antibody present in immune complexes.,online information:C1QC mutation db,PTM:O-linked glycans consist of Glc-Gal disaccharides bound to the oxygen atom of post-translationally added hydroxyl groups.,similarity:Contains 1 C1q domain.,similarity:Contains 1 collagen-like dom
