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You are here:Home » Antibodies » Antibodies-Ion Channel » Anti -CLC1 (Clcn1, Chloride Channel)

Anti -CLC1 (Clcn1, Chloride Channel)


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Clone Host Grade Applications
Polyclonal Rabbit Affinity Purified E B
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.
Catalog #C5837-02K
Rat CLC-1 is 994 aa membrane protein (human CLC-1 988 aa) (1). It is predominantly expressed in skeletal muscles. Defects in CLC1 (CLCN1) are the cause of autosomal recessive generalized myotonia (Becker's disease) (RGM) and autosomal dominant myotonia congenita (Thomsen's disease; MC) which are characterized by skeletal muscle stiffness (delayed relaxation) that is a result of muscle membrane hyperexcitability. .
ApplicationsSuitable for use in ELISA, Western Blotting. Other applications not tested.
Recommended DilutionWestern 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 purified. Control peptide can be used to coat ELISA plates at 1ug/ml.
Optimal dilutions to be determined by the researcher.
Storage and StabilityMay be stored at 4°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°C. Aliquots are stable for at least 12 months at -20°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.
Clone TypePolyclonal
ConcentrationAs reported
FormSupplied as a liquid in PBS, pH 7.4 and 0.1% BSA, 0.1% sodium azide, 40% glycerol.
PurityPurified over the peptide-Sepharose column
ImmunogenA 19 AA Peptide sequence near the N-terminus of rat CLC-1 (1). The peptide was coupled to KLH
SpecificityThe 19 AA rat control peptide 94% conserved in mouse and 84% in 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. The control peptide is available to confirm specificity of antibodies
Important NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.

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