CD46,膜辅蛋白抗体

产品编号HZ-1529R
英文名称CD46
中文名称膜辅蛋白抗体
别 名CD46 antigen; CD46 molecule; CD46 molecule complement regulatory protein; Complement membrane cofactor protein; MCP; Measles virus receptor; Membrane cofactor protein; Membrane cofactor protein precursor; MIC 10; MIC10; TLX; TRA2.10; Trophoblast leucocyte common antigen; Trophoblast lymphocyte cross reactive antigen; Antigen identified by monoclonal antibody TRA 2 10; MCP_HUMAN; membrane cofactor protein isoform 1 precursor.
说 明 书0.1ml 0.2ml
研究领域免疫学 糖蛋白 细胞类型标志物
抗体来源Rabbit
克隆类型Polyclonal
CD46,膜辅蛋白抗体交叉反应Human, Mouse,
产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:20-500 ICC=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43kDa
细胞定位细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human CD46
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.
CD46,膜辅蛋白抗体PubMedPubMed
产品介绍background:
The protein encoded by this gene is a type I membrane protein and is a regulatory part of the complement system. The encoded protein has cofactor activity for inactivation of complement components C3b and C4b by serum factor I, which protects the host cell from damage by complement. In addition, the encoded protein can act as a receptor for the Edmonston strain of measles virus, human herpesvirus-6, and type IV pili of pathogenic Neisseria. Finally, the protein encoded by this gene may be involved in the fusion of the spermatozoa with the oocyte during fertilization. Mutations at this locus have been associated with susceptibility to hemolytic uremic syndrome. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jun 2010].

Function:
Acts as a cofactor for complement factor I, a serine protease which protects autologous cells against complement-mediated injury by cleaving C3b and C4b deposited on host tissue. May be involved in the fusion of the spermatozoa with the oocyte during fertilization. Also acts as a costimulatory factor for T-cells which induces the differentiation of CD4+ into T-regulatory 1 cells. T-regulatory 1 cells suppress immune responses by secreting interleukin-10, and therefore are thought to prevent autoimmunity. A number of viral and bacterial pathogens seem to exploit this property and directly induce an immunosuppressive phenotype in T-cells by binding to CD46.

Subunit:
Interacts with C3b and C4b. Binds to Measles virus H protein, to Human herpesvirus 6 GH protein and to human adenovirus B/D PIV/fiber protein, and acts as a receptor for these viruses. Binds to Streptococcus pyogenes M protein and to type IV pili from Neisseria, and may act as a receptor for these pathogenic bacteria.

Subcellular Location:
Cytoplasmic vesicle, secretory vesicle, acrosome inner membrane; Single-pass type I membrane protein. Note=Inner acrosomal membrane of spermatozoa. Internalized upon binding of Measles virus, Herpesvirus 6 or Neisseria gonorrhoeae, which results in an increased susceptibility of infected cells to complement-mediated injury. In cancer cells or cells infected by Neisseria, shedding leads to a soluble peptide.

Tissue Specificity:
Expressed by all cells except erythrocytes.

CD46,膜辅蛋白抗体Post-translational modifications:
N-glycosylated on Asn-83; Asn-114 and Asn-273 in most tissues, but probably less N-glycosylated in testis. N-glycosylation on Asn-114 and Asn-273 is required for cytoprotective function. N-glycosylation on Asn-114 is required for Measles virus binding. N-glycosylation on Asn-273 is required for Neisseria binding. N-glycosylation is not required for human adenovirus binding.
Extensively O-glycosylated in the Ser/Thr-rich domain. O-glycosylation is required for Neisseria binding but not for Measles virus or human adenovirus binding.
In epithelial cells, isoforms B/D/F/H/J/L/3 are phosphorylated by YES1 in response to infection by Neisseria gonorrhoeae; which promotes infectivity. In T-cells, these isoforms may be phosphorylated by Lck.

DISEASE:
Defects in CD46 are a cause of susceptibility to hemolytic uremic syndrome atypical type 2 (AHUS2) [MIM:612922]. An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Note=Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype. Patients with CD46 mutations seem to have an overall better prognosis compared to patients carrying CFH mutations.

Similarity:
Contains 4 Sushi (CCP/SCR) domains.

Gene ID:
4179

Database links:
Entrez Gene: 4179 Human
Entrez Gene: 17221 Mouse
Entrez Gene: 29333 Rat
Omim: 120920 Human
SwissProt: P15529 Human
SwissProt: O88174 Mouse
SwissProt: Q9Z0M4 Rat
Unigene: 510402 Human
Unigene: 12884 Mouse
Unigene: 163242 Rat

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

MCP为一单链穿膜糖蛋白,是补体系统的一种新的调节蛋白，是内源性辅助因子活性蛋白，属于RCA基因簇的成员。CD46通过GPI锚固定于细胞上。MCP的细胞分布广范，在粒细胞、血小板、T细胞（Th、Ts、Tc）、B细胞、NK细胞、造血细胞系、成纤维细胞、表皮细胞、内皮细胞及星状胶质细胞等都有分布。