Rat Amiloride-sensitive sodium channel subunit beta (SCNN1B) ELISA Kit | SCNN1B elisa kit
Rat Amiloride-sensitive sodium channel subunit beta (SCNN1B) ELISA Kit
Principle of the Assay: SCNN1B ELISA kit applies the competitive enzyme immunoassay technique utilizing an anti-SCNN1B antibody and an SCNN1B-HRP conjugate. The assay sample and buffer are incubated together with SCNN1B-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the SCNN1B concentration since SCNN1B from samples and SCNN1B-HRP conjugate compete for the anti-SCNN1B antibody binding site. Since the number of sites is limited, as more sites are occupied by SCNN1B from the sample, fewer sites are left to bind SCNN1B-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The SCNN1B concentration in each sample is interpolated from this standard curve.
NCBI and Uniprot Product Information
NCBI Description
Nonvoltage-gated, amiloride-sensitive, sodium channels control fluid and electrolyte transport across epithelia in many organs. These channels are heteromeric complexes consisting of 3 subunits: alpha, beta, and gamma. This gene encodes the beta subunit, and mutations in this gene have been associated with pseudohypoaldosteronism type 1 (PHA1), and Liddle syndrome. [provided by RefSeq, Apr 2009]
Uniprot Description
ENaC-beta: Sodium permeable non-voltage-sensitive ion channel inhibited by the diuretic amiloride. Mediates the electrodiffusion of the luminal sodium (and water, which follows osmotically) through the apical membrane of epithelial cells. Controls the reabsorption of sodium in kidney, colon, lung and sweat glands. Also plays a role in taste perception. Defects in SCNN1B are a cause of autosomal recessive pseudohypoaldosteronism type 1 (AR-PHA1). PHA1 is a rare salt wasting disease resulting from target organ unresponsiveness to mineralocorticoids. There are 2 forms of PHA1: the autosomal recessive form that is severe, and the dominant form which is milder and due to defects in mineralocorticoid receptor. AR-PHA1 is characterized by an often fulminant presentation in the neonatal period with dehydration, hyponatraemia, hyperkalaemia, metabolic acidosis, failure to thrive and weight loss. The degree of channel function impairment differentially affects the renin-aldosterone system and urinary Na/K ratios, resulting in distinct genotype-phenotype relationships in PHA1 patients. Loss- of-function mutations are associated with a severe clinical course and age-dependent hyperactivation of the renin-aldosterone system. This feature is not observed in patients with missense mutations that reduce but do not eliminate channel function. Markedly reduced channel activity results in impaired linear growth and delayed puberty. Defects in SCNN1B are a cause of Liddle syndrome (LIDDS). It is an autosomal dominant disorder characterized by pseudoaldosteronism and hypertension associated with hypokalemic alkalosis. The disease is caused by constitutive activation of the renal epithelial sodium channel. Defects in SCNN1B are the cause of bronchiectasis with or without elevated sweat chloride type 1 (BESC1). A debilitating respiratory disease characterized by chronic, abnormal dilatation of the bronchi and other cystic fibrosis-like symptoms in the absence of known causes of bronchiectasis (cystic fibrosis, autoimmune diseases, ciliary dyskinesia, common variable immunodeficiency, foreign body obstruction). Clinical features include sub-normal lung function, sinopulmonary infections, chronic productive cough, excessive sputum production, and elevated sweat chloride in some cases. Belongs to the amiloride-sensitive sodium channel (TC 1.A.6) family. SCNN1B subfamily. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Membrane protein, multi-pass; Membrane protein, integral; Channel, sodium; Transporter, ion channel
Chromosomal Location of Human Ortholog: 16p12.2-p12.1
Cellular Component: integral to plasma membrane; apical plasma membrane; plasma membrane; external side of plasma membrane
Molecular Function: amiloride-sensitive sodium channel activity; protein binding; WW domain binding
Biological Process: sensory perception of taste; response to stimulus; sodium ion transport; sodium ion homeostasis; multicellular organismal water homeostasis; excretion; transmembrane transport
Disease: Bronchiectasis With Or Without Elevated Sweat Chloride 1; Pseudohypoaldosteronism, Type I, Autosomal Recessive; Liddle Syndrome
Research Articles on SCNN1B
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Product Notes
The Rat SCNN1B scnn1b (Catalog #AAA7241883) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7241883 ELISA Kit recognizes Rat SCNN1B. It is sometimes possible for the material contained within the vial of "Amiloride-sensitive sodium channel subunit beta (SCNN1B), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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