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Mouse Anti-BSND Recombinant Antibody (1E8) (CBMAB-A0890-LY)

The product is antibody recognizes BSND. The antibody 1E8 immunoassay techniques such as: WB, ELISA.
See all BSND antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
1E8
Antibody Isotype
IgG2b, κ
Application
WB, E

Basic Information

Immunogen
BSND (NP_476517.1, 221 a.a. ~ 320 a.a) partial recombinant protein with GST tag.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG2b, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.4
Preservative
None
Concentration
Batch dependent
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Bartter syndrome, infantile, with sensorineural deafness (Barttin)
Introduction
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]
Entrez Gene ID
7809
UniProt ID
Q8WZ55
Alternative Names
BART; MGC119283; MGC119284; MGC119285
Function
Functions as a beta-subunit for CLCNKA and CLCNKB chloride channels. In the kidney CLCNK/BSND heteromers mediate chloride reabsorption by facilitating its basolateral efflux. In the stria, CLCNK/BSND channels drive potassium secretion by recycling chloride for the basolateral SLC12A2 cotransporter.
Biological Process
Chloride transport Source: GO_Central
Ion transmembrane transport Source: Reactome
Cellular Location
Cytoplasm; Cell 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).
Involvement in disease
Bartter syndrome 4A, neonatal, with sensorineural deafness (BARTS4A): A form of Bartter syndrome, an autosomal recessive disorder characterized by impaired salt reabsorption in the thick ascending loop of Henle with pronounced salt wasting, hypokalemic metabolic alkalosis, and varying degrees of hypercalciuria. BARTS4A is associated with sensorineural deafness.
Topology
Cytoplasmic: 1-5 aa
Helical: 6-26 aa
Extracellular: 27-32 aa
Helical: 33-53 aa
Cytoplasmic: 54-320 aa
PTM
Palmitoylation is necessary for activation of plasma membrane-inserted CLC-K/barttin channels.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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