CLC5 (Clcn5, Chloride Channel)

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. Rat CLC-5 is 746 aa membrane protein (human CLC-5 746) (1). CLC-5 is abundant in kidney moderate levels are found in the brain, lung, and liver. Mutations or deletions in CLC-5 have been linked with Dent's diseases (excessive urinary calcium, urinary low mol wt proteinuria, and calcium kidney stone), and four related X-linked syndromes of hypercalciuric nephrolithiasis.\n .\nApplications: \nSuitable for use in ELISA and Western Blot. Other applications not tested.\n\nRecommended Dilution:\nELISA: 0.5-1ug/ml using the control peptide to coat plates at 1ug/ml.\nWestern Blot: 1-10ug/ml using ECL. Detects a protein of ~80kD in kidney.\nOptimal dilutions to be determined by the researcher.\n\nRecommended Control Peptide:\nC5837-35: CLC5, Rat (Clcn5, Chloride Channel) Control Peptide\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

C5837-31

Size

50ug

Applications

ELISA, WB

Hosts

Rabbit

Reactivities

Hum, Rat

Form

Supplied as a liquid in PBS, 0.1% BSA.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

References

1. Steinmeyer K et al (1995) J Biol. Chem. 270, 31172-31177; Sakomoto H et al (1996) J Biol. Chem. 271, 10210-10216; Fisher SE et al (1995) Genomics 29, 598-606; Lloyd SE et al (1994) Hum Mol. Genet. 3, 2053-2059; Lloyd SE et al (1996) nature 379, 445\n2. Gunther W et al (1998) PNAS 95, 8075-8080.

Additional Info

Recognizes rat CLC5. No significant sequence homology is detected with other CLCs or other proteins. Sequence Homology: CLC-3, CLC-4 and CLC-5 However, CLC51-P peptide has very little sequence homology with CLC-3 and CLC-4. Species Sequence Homology: mouse, porcine and rabbit-100%, human-92% and guinea pig-84%.