CLC2 (Clcn2, Chloride Channel) (Not for Export EU)
Product Sizes
100ul
£704.00
C5837-05D-100UL
About this Product
- SKU:
- C5837-05D
- Clonality:
- Polyclonal
- Extra Details:
- 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-2 is 887 aa (or 907 aa) membrane protein (human CLC-2 898 aa) (1). Alternatively spliced short form (491 aa) have also been found. CLC-2 is ubiquitously expressed. 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. Applications: Suitable for use in ELISA. Western Blot, though not tested, may potentially be used as an application. Other applications not tested. Recommended Dilution: Western Blot: 1:1000-1:5000 using ECL. Detects multiple CLC-2 bands between 80-90kD bands in various tissues. ELISA: 1:10,000-1:50,000. Control peptide can be used to coat ELISA plates at 1ug/ml. Optimal dilutions to be determined by the researcher. Control Peptide: C5837-05H Storage and Stability: May be stored at 4°C for short-term only. For long-term storage, store 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.
- Host:
- Rabbit
- Immunogen:
- Synthetic peptide consisting of 22aa near the C-terminus of rat CLC-2 (KLH).
- Isotype:
- IgG
- Physical State:
- Supplied as a liquid in 0.05% sodium azide, 40% glycerol.
- Purity:
- Serum
- Shipping Conditions:
- Blue Ice
- Specificity:
- Recognizes Chloride Channel 2. No significant sequence homology is detected with other CLCs or other proteins. Species sequence homology: mouse-100%, porcine, rabbit-95%, human-90%.
- Source:
- rat
- Storage Conditions:
- 4[o]C (-20[o]C Glycerol)
- Supplier:
- United States Biological
- Type:
- Antibodies:Polyclonal Antibody
