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

Chromosome: 3; NC_000003.11 (184063973..184079439, complement). Location: 3q27.1

Synthetic peptide consisting of 22aa near the C-terminus of rat CLC-2. No significant sequence homology is detected with other CLCs or other proteins. Species sequence homology

Highly Purified
Highly purified

Supplied as a liquid in PBS, pH 7.2, 0.1% sodium azide.

May be stored at 4 degree C for short-term only. For long-term storage, store at -20 degree C. Aliquots are stable for at least 6 months at -20 degree 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.

Small volumes of CLCN2 peptide 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.

Control Peptide for C5837-05D (antiserum) and C5837-05E (affinity purified antibody).

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.

Molecular Biology; MB-Ion Channel

ELISA (EL/EIA)

Suitable for use in ELISA, Antibody Blocking. Not suitable for use in Western Blot.
Dilution: Antibody Blocking: 5-10ug per 1ul C5837-05D (antiserum) or per 1ug C5837-05E (affinity purified antibody).
ELISA: 0.5-1ug/ml. Control peptide can be used to coat ELISA plates at 1ug/ml.

98,535 Da[Similar Products]

chloride channel, voltage-sensitive 2

chloride channel protein 2; chloride channel 2

Chloride channel protein 2

ClC-2??[Similar Products]

CLCN2_HUMAN

This gene encodes a voltage-gated chloride channel. The encoded protein is a transmembrane protein that maintains chloride ion homeostasis in various cells. Defects in this gene may be a cause of certain epilepsies. Four transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2012]

Function: Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. Ref.7 Ref.11

Subcellular location: Membrane; Multi-pass membrane protein.

Tissue specificity: Ubiquitously expressed. Moderately expressed in aortic and coronary vascular smooth muscle cells and expressed at a low level in aortic endothelial cells. Ref.6

Involvement in disease: Epilepsy, idiopathic generalized 11 (EIG11) [MIM:607628]: A disorder characterized by recurring generalized seizures in the absence of detectable brain lesions and/or metabolic abnormalities. Generalized seizures arise diffusely and simultaneously from both hemispheres of the brain.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry.Juvenile absence epilepsy 2 (JAE2) [MIM:607628]: A subtype of idiopathic generalized epilepsy characterized by onset occurring around puberty, absence seizures, generalized tonic-clonic seizures (GTCS), GTCS on awakening, and myoclonic seizures.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry.Juvenile myoclonic epilepsy 8 (EJM8) [MIM:607628]: A subtype of idiopathic generalized epilepsy. Patients have afebrile seizures only, with onset in adolescence (rather than in childhood) and myoclonic jerks which usually occur after awakening and are triggered by sleep deprivation and fatigue.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry.

Miscellaneous: The CLC channel family contains both chloride channels and proton-coupled anion transporters that exchange chloride or another anion for protons. The absence of conserved gating glutamate residues is typical for family members that function as channels

By similarity.

Sequence similarities: Belongs to the chloride channel (TC 2.A.49) family. ClC-2/CLCN2 subfamily. [View classification]Contains 2 CBS domains.

Sequence caution: The sequence AAH21578.1 differs from that shown. Reason: Erroneous translation. Wrong choice of frame.

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