Anti -CLC1 (Clcn1, Chloride Channel)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 7; NC_000007.13 (143013219..143049097). Location: 7q32-qter; 7q35

Polyclonal

IgG

Rabbit

Recognizes rat CLC1. No significant sequence homology is detected with other CLCs or other proteins.

Serum
Serum

Supplied as a liquid in PBS, 0.05% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree 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.

Small volumes of anti-CLCN1 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.

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-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.

Antibodies; Abs to Ion Channel

ELISA (EL/EIA), Western Blot (WB)

Dilution: Western Blot: 1:1000-1:5000
ELISA: 1:10,000-50,000. C5837-02E Control peptide can be used to coat ELISA plates at 1ug/ml.

108,626 Da[Similar Products]

chloride channel 1, skeletal muscle

chloride channel protein 1; clC-1; OTTHUMP00000202223; chloride channel protein, skeletal muscle

Chloride channel protein 1

CLC1??[Similar Products]

CLCN1_HUMAN

The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite diverse functional characteristics while sharing significant sequence homology. The protein encoded by this gene regulates the electric excitability of the skeletal muscle membrane. Mutations in this gene cause two forms of inherited human muscle disorders: recessive generalized myotonia congenita (Becker) and dominant myotonia (Thomsen). [provided by RefSeq]

CLCN1: Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. Defects in CLCN1 are the cause of Thomsen disease (THD); also known as autosomal dominant myotonia congenita (MCD). THD is characterized by skeletal muscle stiffness (delayed relaxation), due to membrane hyperexcitability. A variant form of Thomsen disease is myotonia levior that is characterized by milder symptoms, later onset and absence of muscle hypo- and hypertrophy. Defects in CLCN1 are the cause of autosomal recessive myotonia congenita (MCR); also known as Becker disease. Belongs to the chloride channel (TC 2.A.49) family. ClC-1/CLCN1 subfamily.

Protein type: Membrane protein, integral; Transporter, ion channel; Membrane protein, multi-pass; Transporter; Channel, chloride

Chromosomal Location of Human Ortholog: 7q35

Cellular Component: integral to plasma membrane; plasma membrane; sarcolemma

Molecular Function: chloride channel activity; voltage-gated chloride channel activity

Biological Process: muscle contraction; transport; action potential propagation; transmembrane transport

Disease: Myotonia Congenita, Autosomal Recessive; Myotonia Congenita, Autosomal Dominant

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