Signal Transduction Antibodies 1

Anti-SCNN1B Antibody (CAB12699)

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SKU:
CAB12699
Product Type:
Antibody
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Signal Transduction

Description

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Antibody Name:Anti-SCNN1B Antibody
Antibody SKU:CAB12699
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Mouse, Rat
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 1:1000 - 1:2000
Reactivity:Mouse, Rat
Positive Samples:Mouse kidney, Rat kidney
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
Anti-SCNN1B Antibody (CAB12699)Western blot analysis of extracts of various cell lines, using SCNN1B antibody (CAB12699) 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: 10s.
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