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Rabbit Anti-LRRC8A  antibody (bs-18409R)  
~~~促銷代碼KT202411~~~
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說明書: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價(jià)
產(chǎn)品編號 bs-18409R
英文名稱 Rabbit Anti-LRRC8A  antibody
中文名稱 富含亮氨酸重復(fù)蛋白8A抗體
別    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.  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  淋巴細(xì)胞  b-淋巴細(xì)胞  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Dog,Horse)
產(chǎn)品應(yīng)用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 94kDa
細(xì)胞定位 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LRRC8A: 84-180/810 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
Q8IWT6

Gene ID:
52626

Database links:

Entrez Gene: 56262 Human

Entrez Gene: 505605 Cow

Entrez Gene: 491309 Dog

Entrez Gene: 241296 Mouse

Entrez Gene: 311846 Rat

Omim: 608360 Human

SwissProt: Q8IWT6 Human

SwissProt: Q80WG5 Mouse

SwissProt: Q4V8I7 Rat

Unigene: 643600 Human

Unigene: 389232 Mouse

Unigene: 475219 Mouse

Unigene: 33512 Rat



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