富含亮氨酸重复蛋白8A抗体
规格：1mg/1ml
英文名： LRRC8A
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.
分子量： 94kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human LRRC8A
交叉反应：Human, Mouse, Rat, Dog, Pig, Horse, Rabbit, Sheep,
细胞定位：细胞膜
富含亮氨酸重复蛋白8A抗体产品介绍：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,富含亮氨酸重复蛋白8A抗体 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,富含亮氨酸重复蛋白8A抗体 resulting in deletion of the eighth, ninth, and half of the seventh LRR domains. Similarity: Contains 17 LRR (leucine-rich) repeats. 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 MouseSwissProt: Q4V8I7 Rat Unigene: 643600 Human Unigene: 389232 Mouse Unigene: 475219 Mouse Unigene: 33512 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品应用：富含亮氨酸重复蛋白8A抗体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.
研究领域：细胞生物 **学 **细胞 b-**细胞
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid