C4b-A,补体C4b-A蛋白抗体

产品编号HZ-10452R
英文名称C4b-A
中文名称补体C4b-A蛋白抗体
别 名C4b-A; complement C4-A proprotein; Acidic complement C4; Basic complement C4; CH; Chido blood group; Complement component 4A; Complement component 4B; RG; Rodgers blood group; CO4A_HUMAN; CO4B_HUMAN; C4A; C4; C4A2; C4A3; C4A4; C4A6; C4AD; C4S; CO4; CPAMD2; RG.
说 明 书0.1ml 0.2ml
研究领域
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human,
C4b-A,补体C4b-A蛋白抗体产品应用ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量76/190kDa
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human C4b-A
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
C4b-A,补体C4b-A蛋白抗体PubMedPubMed
产品介绍background:
This gene encodes the acidic form of complement factor 4, part of the classical activation pathway. The protein is expressed as a single chain precursor which is proteolytically cleaved into a trimer of alpha, beta, and gamma chains prior to secretion. The trimer provides a surface for interaction between the antigen-antibody complex and other complement components. The alpha chain may be cleaved to release C4 anaphylatoxin, a mediator of local inflammation. Deficiency of this protein is associated with systemic lupus erythematosus and type I diabetes mellitus. This gene localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. Varying haplotypes of this gene cluster exist, such that individuals may have 1, 2, or 3 copies of this gene. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Nov 2011].

Function:
Non-enzymatic component of C3 and C5 convertases and thus essential for the propagation of the classical complement pathway. Covalently binds to immunoglobulins and immune complexes and enhances the solubilization of immune aggregates and the clearance of IC through CR1 on erythrocytes. C4A isotype is responsible for effective binding to form amide bonds with immune aggregates or protein antigens, while C4B isotype catalyzes the transacylation of the thioester carbonyl group to form ester bonds with carbohydrate antigens.
Derived from proteolytic degradation of complement C4, C4a anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes.

Subunit:
Circulates in blood as a disulfide-linked trimer of an alpha, beta and gamma chain.

C4b-A,补体C4b-A蛋白抗体Subcellular Location:
Secreted.

Tissue Specificity:
Complement component C4 is expressed at highest levels in the liver, at moderate levels in the adrenal cortex, adrenal medulla, thyroid gland,and the kidney, and at lowest levels in the heart, ovary, small intestine, thymus, pancreas and spleen. The extra-hepatic sites of expression may be important for the local protection and inflammatory response.

Post-translational modifications:
Prior to secretion, the single-chain precursor is enzymatically cleaved to yield non-identical chains alpha, beta and gamma. During activation, the alpha chain is cleaved by C1 into C4a and C4b, and C4b stays linked to the beta and gamma chains. Further degradation of C4b by C1 into the inactive fragments C4c and C4d blocks the generation of C3 convertase. The proteolytic cleavages often are incomplete so that many structural forms can be found in plasma.
N- and O-glycosylated. O-glycosylated with a core 1 or possibly core 8 glycan.

DISEASE:
Defects in C4A are the cause of complement component 4A deficiency (C4AD) [MIM:614380]. A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus with or without associated glomerulonephritis.
Defects in C4A are a cause of susceptibility to systemic lupus erythematosus (SLE) [MIM:152700]. A chronic, inflammatory and often febrile multisystemic disorder of connective tissue. It affects principally the skin, joints, kidneys and serosal membranes. It is thought to represent a failure of the regulatory mechanisms of the autoimmune system. Note=Interindividual copy-number variation (CNV) of complement component C4 and associated polymorphisms result in different susceptibilities to SLE. The risk of SLE susceptibility has been shown to be significantly increased among subjects with only two copies of total C4. A high copy number is a protective factor against SLE.

Similarity:
Contains 1 anaphylatoxin-like domain.
Contains 1 NTR domain.

Gene ID:
720

Database links:
Entrez Gene: 100293534 Human
Entrez Gene: 100507685 Human
Entrez Gene: 720 Human
Entrez Gene: 721 Human
Omim: 120810 Human
SwissProt: P0C0L4 Human
SwissProt: P0C0L5 Human
Unigene: 534847 Human
Unigene: 720022 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.