The CLCNKB Polyclonal Antibody (PACO57704) is a valuable tool for researchers studying CLCNKB, a key protein involved in chloride transport and kidney function. This antibody, produced in rabbits, exhibits high reactivity with human samples and is validated for use in Western blotting and immunohistochemistry applications. By binding to CLCNKB, this antibody allows for precise detection and analysis of the protein in various cell types, making it an essential component for studies in nephrology and related fields.
CLCNKB is essential for maintaining chloride balance in the kidney and is implicated in various kidney disorders, including Bartter syndrome. Understanding the role and regulation of CLCNKB is crucial for developing targeted therapies for kidney diseases and improving patient outcomes. With its high specificity and reliability, the CLCNKB Polyclonal Antibody is a valuable tool for researchers investigating the function and dysfunction of CLCNKB in health and disease.
Western Blot. Positive WB detected in: Hela whole cell lysate, Rat kidney tissue. All lanes: CLCNKB antibody at 3µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 76, 57 kDa. Observed band size: 76 kDa.
Immunofluorescence staining of HepG2 cells with PACO57704 at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
IHC image of PACO57704 diluted at 1:300 and staining in paraffin-embedded human breast cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
Background:
Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. May be important in urinary concentrating mechanisms.
Synonyms:
Chloride channel protein ClC-Kb (Chloride channel Kb) (ClC-K2), CLCNKB
UniProt Protein Function:
CLCNKB: Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. May be important in urinary concentrating mechanisms. Defects in CLCNKB are the cause of Bartter syndrome type 3 (BS3); also known as classic Bartter syndrome. It is an autosomal recessive form of often severe intravascular volume depletion due to renal salt-wasting associated with low blood pressure, hypokalemic alkalosis, hypercalciuria, and normal serum magnesium levels. Defects in CLCNKB are a cause of Bartter syndrome type 4B (BS4B). A digenic, 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. Bartter syndrome type 4B is associated with sensorineural deafness. Belongs to the chloride channel (TC 2.A.49) family. CLCNKB subfamily.Protein type: Membrane protein, multi-pass; Transporter, ion channel; Membrane protein, integral; TransporterChromosomal Location of Human Ortholog: 1p36Cellular Component: integral to plasma membrane; plasma membraneMolecular Function: voltage-gated chloride channel activityBiological Process: excretion; transportDisease: Bartter Syndrome, Type 3; Bartter Syndrome, Type 4b
UniProt Protein Details:
NCBI Summary:
The protein encoded by this gene is a member of the family of voltage-gated chloride channels. Chloride channels have several functions, including the regulation of cell volume, membrane potential stabilization, signal transduction and transepithelial transport. This gene is expressed predominantly in the kidney and may be important for renal salt reabsorption. Mutations in this gene are associated with autosomal recessive Bartter syndrome type 3 (BS3). Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2009]