CLC1, Rat (Clcn1, Chloride Channel) Control Peptide
Biozol Catalog Number:
USB-C5837-02E
Supplier Catalog Number:
C5837-02E
Alternative Catalog Number:
USB-C5837-02E-100
Manufacturer:
US Biological
Category:
Molekularbiologie
Application:
ELISA, WB
An 18aa Peptide sequence near the C-terminus of rat CLC-1. 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 (Thomsens disease), cystic fibrosis, Bartters syndrome type III, Dents 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-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 (Beckers disease) (RGM) and autosomal dominant myotonia congenita (Thomsens disease, MC) which are characterized by skeletal muscle stiffness (delayed relaxation) that is a result of muscle membrane hyperexcitability.
Purity:
Highly purified
Form:
Supplied as a liquid in PBS, pH 7.2.
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