Price range: $268.00 through $328.00
Background:This gene encodes an essential beta subunit for CLC chloride channels. These heteromeric channels localize to basolateral membranes of renal tubules and of potassium-secreting epithelia of the inner ear. Mutations in this gene have been associated with Bartter syndrome with sensorineural deafness. [provided by RefSeq, Jul 2008]
| Size | 50μL/100μL |
| Target | BSND |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC |
| MW(Calculated) | 35kD |
| MW(Observed) | 35kD |
| Host Species | Rabbit |
| Isotype | IgG,Kappa |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | IHC 1:200-1:1000; WB 1:1000-1:5000; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Purification | Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | RM1122R |
| Immunogen | The specific immunogen used to produce this antibody is proprietary information. |
| Specificity | Endogenous |
| Gene Name | BSND,BART |
| Protein Name | Barttin |
| Other Name | BART |
| Species | Human |
| Gene ID | 7809 |
| Uniprot | Q8WZ55, |
| Species.1 | Mouse |
| Gene ID.1 | 140475 |
| Uniprot.1 | Q8VIM4 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | SUBCELLULAR LOCATION: Basolateral cell membrane {ECO:0000269|PubMed:18776122}; Multi-pass membrane protein {ECO:0000255}. Note=Mostly sorted at the basolateral membrane. A significant amount also observed intracellularly. Staining in membranes of the renal tubule and of potassium-secreting epithelia of the inner ear is basolateral (By similarity). {ECO:0000250}. |
| Tissue Expression | TISSUE SPECIFICITY: Expressed primarily in kidney. Expressed in specific nephron segments and in the stria vascularis of the inner ear. {ECO:0000269|PubMed:11687798}. |
| Signaling_pathway | |
| Research Areas | |
| Function | FUNCTION: Regulatory subunit of anion-selective CLCNKA:BSND and CLCNKB:BSND heteromeric channels involved in basolateral chloride conductance along the nephron to achieve urine concentration and maintain systemic acid-base homeostasis, and in the stria vascularis of the inner ear to establish the endocochlear potential necessary for normal hearing (PubMed:11734858, PubMed:12111250, PubMed:12574213, PubMed:16849430, PubMed:18776122, PubMed:19646679, PubMed:20538786, PubMed:26013830). Most likely acts as a chaperone that allosterically regulates proper sorting of CLCNKA:BSND and CLCNKB:BSND channels at the basolateral plasma membrane domain and functional switch to ion conducting state. Mediates constitutive opening of channel common gates (PubMed:11734858, PubMed:12111250, PubMed:12574213, PubMed:16849430, PubMed:18776122, PubMed:19646679, PubMed:20538786, PubMed:26013830). {ECO:0000269|PubMed:11734858, ECO:0000269|PubMed:12111250, ECO:0000269|PubMed:12574213, ECO:0000269|PubMed:16849430, ECO:0000269|PubMed:18776122, ECO:0000269|PubMed:19646679, ECO:0000269|PubMed:20538786, ECO:0000269|PubMed:26013830}. |
