Western Blot (WB)
(Western blot analysis of extracts of PC-3 cell lines, using SCNN1B antibody.)
Rabbit SCNN1B Polyclonal Antibody | anti-SCNN1B antibody
SCNN1B antibody
Western Blot (WB)
(Western blot analysis of extracts of PC-3 cell lines, using SCNN1B antibody.)
Western Blot (WB)
(Western blot analysis of extracts of PC-3 cell lines, using SCNN1B antibody.)
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: Transporter, ion channel; Membrane protein, multi-pass; Channel, sodium; Membrane protein, integral
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 SCNN1B scnn1b (Catalog #AAA9412270) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The SCNN1B antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's SCNN1B can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Western blotting: 1:500 - 1:2000. Researchers should empirically determine the suitability of the SCNN1B scnn1b for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "SCNN1B, Polyclonal Antibody" 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
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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