Product Name
Barttin (BSND), Polyclonal Antibody
Full Product Name
Anti - Barttin
Product Synonym Names
Barttin
Product Gene Name
anti-BSND antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Chromosome Location
Chromosome: 1; NC_000001.10 (55464617..55474465). Location: 1p32.1
3D Structure
ModBase 3D Structure for Q5VU50
Host
Host: Rabbit
Source: Human
Specificity
The control peptide 100% conserved in mouse, rat and human CLC-1. No significant sequence homology is detected with other CLCs or other proteins. Because of the low MW (<3kD) of the control peptide, it is not recommended for Western. Control peptide, because of its low MW (<3kD), is not suitable for Western. It should be used for ELISA or antibody blocking experiments (use 5-10 ug control peptide per 1 ug of aff pure IgG or 1 ul antiserum) to confirm antibody specificity
Purity/Purification
Purified by immunoaffinity chromatography.
Form/Format
Affinity Purified
Supplied as a liquid in PBS, pH 7.4 and 0.1% sodium azide, 40% glycerol.
Immunogen
A 16-aa Peptide sequence after the 2nd TM domain and in the cytoplasmic region of the human/mouse/rat Barttin (1) was synthesized, coupled to KLH
Preparation and Storage
May be stored at 4 degree C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and add glycerol (40-50%). Freeze at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Other Notes
Small volumes of anti-BSND antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-BSND antibody
Chloride is a critical component of all living cells. Voltage-gated chloride channels regulate cellular traffic of chloride ion. The chloride channels (CIC or CLC) performs several functions including the regulation of cell volume, membrane potential stabilization, signal transduction, and transepithelial transport. Mutations in CIC genes have been linked with several human diseases including myotonias (Thomsen's disease), cystic fibrosis, Bartters syndrome type III, Dent's disease, and X-linked recessive nephrolithiasis. In mammals, CLC proteins form a superfamily of at least 9 different genes (CLC1-7 also known as CLCN1-7 and CLK1-2 or CLCKa and CLCKb). Additional forms of these proteins are obtained by alternative splicing. All CLC proteins (~700-1000 aa) are predicted to contain 10 (possibly 12) transmembrane domains. Except CLC-1 and CLC-K1/K2 that are specific for kidney, most other CLC are widely distributed in various tissues.
Product Categories/Family for anti-BSND antibody
Abs to Ion Channel
Applications Tested/Suitable for anti-BSND antibody
ELISA, Western Blot
Application Notes for anti-BSND antibody
Suitable for use in ELISA, Western Blotting. Other applications not tested.
Dilution: Western Blot: 1:1000-1:5000 for neat serum and 1-10ug/ml for affinity pure antibody using ECL.
ELISA: 1:10,000-50,000 for neat serum and 0.5-1ug/ml for affinity pure. Control peptide can be used to coat ELISA plates at 1ug/ml.
Optimal dilutions to be determined by the researcher.
NCBI/Uniprot data below describe general gene information for BSND. It may not necessarily be applicable to this product.
NCBI Accession #
NP_476517.1
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NCBI GenBank Nucleotide #
NM_057176.2
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UniProt Primary Accession #
Q5VU50
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UniProt Related Accession #
Q5VU50; Q8WZ55[Other Products]
Molecular Weight
35,197 Da[Similar Products]
NCBI Official Full Name
barttin
NCBI Official Synonym Full Names
Bartter syndrome, infantile, with sensorineural deafness (Barttin)
NCBI Official Symbol
BSND??[Similar Products]
NCBI Official Synonym Symbols
BART; DFNB73; MGC119283; MGC119284; MGC119285
??[Similar Products]
NCBI Protein Information
barttin; OTTHUMP00000009804; deafness, autosomal recessive 73
UniProt Protein Name
Bartter syndrome, infantile, with sensorineural deafness (Barttin)
UniProt Gene Name
BSND??[Similar Products]
UniProt Entry Name
Q5VU50_HUMAN
Product References and Citations for anti-BSND antibody
??1. Birkenhager R et al (2001) Nat. genet. 29, 310-324; Estevez R et al (2001) Nature 414, 558-561; Brennan TM et al (1998) Am. J. Hum. Genet. 62, 355-361
Precautions
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Disclaimer
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