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LRRC8A Antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, IHC, E |
|---|---|
| Primary Accession | Q8IWT6 |
| Other Accession | NP_062540, 62241040 |
| Reactivity | Human, Mouse, Rat |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | IgG |
| Calculated MW | Predicted: 89 kDa; Observed: 95 kDa |
| Application Notes | LRRC8A antibody can be used for detection of LRRC8A by Western blot at 1 - 2 µg/ml. Antibody can also be used for immunohistochemistry starting at 2.5 µg/mL. |
| Gene ID | 56262 |
|---|---|
| Target/Specificity | LRRC8A; LRRC8A antibody is human, mouse and rat reactive. |
| Reconstitution & Storage | LRRC8A antibody can be stored at 4℃ for three months and -20℃, stable for up to one year. |
| Precautions | LRRC8A Antibody is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | LRRC8A {ECO:0000303|PubMed:22532330, ECO:0000312|HGNC:HGNC:19027} |
|---|---|
| Function | Essential component of the volume-regulated anion channel (VRAC, also named VSOAC channel), an anion channel required to maintain a constant cell volume in response to extracellular or intracellular osmotic changes (PubMed:24725410, PubMed:29769723, PubMed:24790029, PubMed:26530471, PubMed:26824658, PubMed:28193731). The VRAC channel conducts iodide better than chloride and can also conduct organic osmolytes like taurine (PubMed:24725410, PubMed:30095067, PubMed:24790029, PubMed:26530471, PubMed:26824658, PubMed:28193731). Mediates efflux of amino acids, such as aspartate and glutamate, in response to osmotic stress (PubMed:28193731). LRRC8A and LRRC8D are required for the uptake of the drug cisplatin (PubMed:26530471). In complex with LRRC8C or LRRC8E, acts as a transporter of immunoreactive cyclic dinucleotide GMP-AMP (2'-3'-cGAMP), an immune messenger produced in response to DNA virus in the cytosol: mediates both import and export of 2'-3'-cGAMP, thereby promoting transfer of 2'-3'-cGAMP to bystander cells (PubMed:33171122). In contrast, complexes containing LRRC8D inhibit transport of 2'-3'-cGAMP (PubMed:33171122). Required for in vivo channel activity, together with at least one other family member (LRRC8B, LRRC8C, LRRC8D or LRRC8E); channel characteristics depend on the precise subunit composition (PubMed:24790029, PubMed:26824658, PubMed:28193731). Can form functional channels by itself (in vitro) (PubMed:26824658). Involved in B-cell development: required for the pro-B cell to pre-B cell transition (PubMed:14660746). Also required for T-cell development (By similarity). Required for myoblast differentiation: VRAC activity promotes membrane hyperpolarization and regulates insulin-stimulated glucose metabolism and oxygen consumption (By similarity). Also acts as a regulator of glucose-sensing in pancreatic beta cells: VRAC currents, generated in response to hypotonicity- or glucose-induced beta cell swelling, depolarize cells, thereby causing electrical excitation, leading to increase glucose sensitivity and insulin secretion (PubMed:29371604). Also plays a role in lysosome homeostasis by forming functional lysosomal VRAC channels in response to low cytoplasmic ionic strength condition: lysosomal VRAC channels are necessary for the formation of large lysosome-derived vacuoles, which store and then expel excess water to maintain cytosolic water homeostasis (PubMed:31270356, PubMed:33139539). |
| Cellular Location | Cell membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein. Note=Mainly localizes to the cell membrane, with some intracellular localization to lysosomes |
| Tissue Location | 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. |
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Provided below are standard protocols that you may find useful for product applications.
Background
The leucine rich repeat containing 8 family member A protein (LRRC8A) belongs the LRRC8 family of proteins within the LRR superfamily of proteins, which are involved in diverse biological processes, including cell adhesion, cellular trafficking, and hormone-receptor interactions (1,2). LRRC8A is a 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 (1). LRRC8A has also been shown to be an essential component of the volume-regulated anion channel VRAC (3).
References
Sawada A, Takihara Y, Kim JY, et al. A congenital mutation of the novel gene LRRC8 causes agammaglobulinemia in humans. J. Clin. Invest. 2003; 112:1707-13.
Kubota K, Kim JY, Sawada A, et al. LRRC8 involved in B cell development belongs to a novel family of leucine-rich repeat proteins. FEBS Lett. 2004; 564:147-52.
Voss FK, Ullrich F, Munch J, et al. Identification of LRRC8 heteromers as an essential component of the volume-regulated anion channel VRAC. Science 2014; 344:634-8.
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