Anti-SCNN1B Antibody (CAB1765)
- SKU:
- CAB1765
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Signal Transduction
Frequently bought together:
Description
| Antibody Name: | Anti-SCNN1B Antibody |
| Antibody SKU: | CAB1765 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Human, Mouse |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 406-640 of human SCNN1B (NP_000327.2). |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Human, Mouse |
| Positive Samples: | 293T, LO2, Mouse lung |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 406-640 of human SCNN1B (NP_000327.2). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | YPLP RGEK YCNN RDFP DWAH CYSD LQMS VAQR ETCI GMCK ESCN DTQY KMTI SMAD WPSE ASED WIFH VLSQ ERDQ STNI TLSR KGIV KLNI YFQE FNYR TIEE SAAN NIVW LLSN LGGQ FGFW MGGS VLCL IEFG EIII DFVW ITII KLVA LAKS LRQR RAQA SYAG PPPT VAEL VEAH TNFG FQPD TAPR SPNT GPYP SEQA LPIP GTPP PNYD SLRL QPLD VIES DSEG DAI |
| Gene ID: | 6338 |
| Uniprot: | P51168 |
| Cellular Location: | Apical cell membrane, Cytoplasmic vesicle membrane, Multi-pass membrane protein |
| Calculated MW: | 72kDa/77kDa |
| Observed MW: | 75kDa |
| Synonyms: | SCNN1B, BESC1, ENaCb, ENaCbeta, SCNEB |
| Background: | 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. |
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Protein type:Membrane protein, integral; Channel, sodium; Membrane protein, multi-pass; 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 |
| NCBI Summary: | 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 Code: | P51168 |
| NCBI GenInfo Identifier: | 8928561 |
| NCBI Gene ID: | 6338 |
| NCBI Accession: | P51168.2 |
| UniProt Secondary Accession: | P51168,O60891, Q96KG2, Q9UJ32, Q9UMU5, C5HTZ2, |
| UniProt Related Accession: | P51168 |
| Molecular Weight: | 640 |
| NCBI Full Name: | Amiloride-sensitive sodium channel subunit beta |
| NCBI Synonym Full Names: | sodium channel, non-voltage-gated 1, beta subunit |
| NCBI Official Symbol: | SCNN1B |
| NCBI Official Synonym Symbols: | BESC1; ENaCb; SCNEB; ENaCbeta |
| NCBI Protein Information: | amiloride-sensitive sodium channel subunit beta; beta-ENaC; beta-NaCH; epithelial Na(+) channel subunit beta; epithelial sodium channel beta-2 subunit; epithelial sodium channel beta-3 subunit; sodium channel, nonvoltage-gated 1, beta; nasal epithelial sodium channel beta subunit; nonvoltage-gated sodium channel 1 subunit beta; amiloride-sensitive sodium channel subunit beta 1 |
| UniProt Protein Name: | Amiloride-sensitive sodium channel subunit beta |
| UniProt Synonym Protein Names: | Beta-NaCH; Epithelial Na(+) channel subunit beta; Beta-ENaC; ENaCB; Nonvoltage-gated sodium channel 1 subunit beta; SCNEB |
| Protein Family: | Amiloride-sensitive sodium channel |
| UniProt Gene Name: | SCNN1B |
| UniProt Entry Name: | SCNNB_HUMAN |
 | Western blot analysis of extracts of various cell lines, using SCNN1B antibody (CAB1765) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s. |
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