Product Name	Anti-LRRC8A/AF594
Chinese Name	AF594标记的富含亮氨酸重复蛋白8A抗体
Alias	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.
Research Area	Cell biology  immunology  lymphocyte  b-lymphocyte
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species
Applications	ICC=1:50-200 IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	94kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human LRRC8A
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	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.
Product Detail	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: 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.

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