Complement factor 8 beta,补体C8β链抗体

产品编号HZ-13969R
英文名称Complement factor 8 beta
中文名称补体C8β链抗体
别 名C8b; CO8B_HUMAN; Complement component 8 beta polypeptide; Complement component 8 subunit beta; Complement component C8 beta chain.
说 明 书0.1ml 0.2ml
研究领域细胞生物 细菌及病毒 细胞膜蛋白
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Rabbit,
Complement factor 8 beta,补体C8β链抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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61kDa
细胞定位细胞浆
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human Complement factor 8 beta
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.
Complement factor 8 beta,补体C8β链抗体PubMedPubMed
产品介绍background:
This gene encodes one of the three subunits of the complement component 8 (C8) protein. C8 is composed of equimolar amounts of alpha, beta and gamma subunits, which are encoded by three separate genes. C8 is one component of the membrane attack complex, which mediates cell lysis, and it initiates membrane penetration of the complex. This protein mediates the interaction of C8 with the C5b-7 membrane attack complex precursor. In humans deficiency of this protein is associated with increased risk of meningococcal infections. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2013]

Function:
Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells.

Subcellular Location:
Secreted.

Tissue Specificity:

Post-translational modifications:
N-glycosylated; contains one or two bound glycans. Not O-glycosylated.

DISEASE:
Defects in C8B are a cause of complement component 8 deficiency type 2 (C8D2) [MIM:120960]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.

Similarity:
Belongs to the complement C6/C7/C8/C9 family.
Contains 1 EGF-like domain.
Contains 1 LDL-receptor class A domain.
Contains 1 MACPF domain.
Contains 2 TSP type-1 domains.

Gene ID:
732

Database links:
Entrez Gene: 732 Human
Entrez Gene: 100009385 Rabbit
Omim: 120960 Human
SwissProt: P07358 Human
SwissProt: P98137 Rabbit
Unigene: 391835 Human

Complement factor 8 beta,补体C8β链抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.