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货号： bs-18409R-AF555 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-18409R-AF555
- 英文名称
- Anti-LRRC8A/AF555
- 中文名称
- AF555标记的富含亮氨酸重复蛋白8A抗体
- 别 名
- AGM5; FLJ10337; FLJ41617; KIAA1437; Leucine rich repeat containing 8 family member A; Leucine rich repeat containing protein 8A; Leucine-rich repeat-containing protein 8A; LRC8A_HUMAN; LRRC8; Lrrc8a.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 免疫学 淋巴细胞 b-淋巴细胞
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Horse, Rabbit, Sheep,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 94kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human LRRC8A
- 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.
- 产品介绍
- background:
This gene encodes a protein belonging to the leucine-rich repeat family of proteins, which are involved in diverse biological processes, including cell adhesion, cellular trafficking, and hormone-receptor interactions. This family member is a putative four-pass transmembrane protein that plays a role in B cell development. Defects in this gene cause autosomal dominant non-Bruton type agammaglobulinemia, an immunodeficiency disease resulting from defects in B cell maturation. Multiple alternatively spliced transcript variants, which encode the same protein, have been identified for this gene. [provided by RefSeq, Jul 2008]
Function:
Involved in B-cell development. Required for the pro-B cell to pre-B cell transition.
Subcellular Location:
Membrane.
Tissue Specificity:
Expressed in brain, kidney, ovary, lung, liver, heart, and fetal brain and liver. Found at high levels in bone marrow; lower levels are detected in peripheral blood cells. Expressed on T-cells as well as on B-lineage cells.
DISEASE:
Defects in LRRC8A are the cause of agammaglobulinemia type 5 (AGM5) [MIM:613506]. It is a primary immunodeficiency characterized by profoundly low or absent serum antibodies and low or absent circulating B-cells due to an early block of B-cell development. Affected individuals develop severe infections in the first years of life. Note=A chromosomal aberration involving LRRC8 has been found in a patient with congenital agammaglobulinemia. Translocation t(9;20)(q33.2;q12). The translocation truncates the LRRC8 gene, resulting in deletion of the eighth, ninth, and half of the seventh LRR domains.
Similarity:
Contains 17 LRR (leucine-rich) repeats.
Database links:Entrez Gene: 56262Human
Entrez Gene: 505605Cow
Entrez Gene: 491309Dog
Entrez Gene: 241296Mouse
Entrez Gene: 311846Rat
Omim: 608360Human
SwissProt: Q8IWT6Human
SwissProt: Q80WG5MouseSwissProt: Q4V8I7Rat
Unigene: 643600Human
Unigene: 389232Mouse
Unigene: 475219Mouse
Unigene: 33512Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.