Anti -CLC2 (Clcn2, Chloride Channel)

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

Chromosome: 3; NC_000003.11 (184063976..184079391, complement). Location: 3q27-q28

Polyclonal

IgG

Rabbit

Recognizes rat Chloride Channel 2. No significant sequence homology is detected with other CLCs or other proteins. Species Sequence Homology

Affinity Purified
Purified by immunoaffinity chromatography.

Supplied as a liquid in PBS, 0.1% BSA, 40% glycerol.

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 12 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 anti-CLCN2 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 ions. 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 (~700-1000aa) 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 887aa (or 907aa) membrane protein (human CLC-2 898aa) (1). An alternatively spliced short form (491aa) has 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.

Antibodies; Abs to Ion Channel

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

Suitable for use in ELISA, Immunohistochemistry and Western Blot.
Dilution: ELISA: 0.5-1ug/ml. Control peptide can be used to coat ELISA plates at 1ug/ml.
Immunohistochemistry: 1-20ug/ml in paraformaldehyde fixed sections of tissues.
Western Blot: 1-10ug/ml using ECL. An antibody made to the C-terminal region has detected multiple CLC-2 bands between 80-90kD bands in various tissues (7,8).

98,535 Da[Similar Products]

chloride channel 2

chloride channel protein 2; clC-2; OTTHUMP00000210513; OTTHUMP00000210514; OTTHUMP00000210515; OTTHUMP00000210516

Chloride channel protein 2

CLCN2_HUMAN

The transmembrane protein encoded by this gene is a voltage-gated chloride channel 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]

CLCN2: 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 CLCN2 are associated with susceptibility to epilepsy, idiopathic generalized type 11 (EIG11). 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. Defects in CLCN2 are associated with juvenile absence epilepsy type 2 (JAE2). JAE is a subtype of idiopathic generalized epilepsy (IGE) characterized by onset occurring around puberty, absence seizures, generalized tonic- clonic seizures (GTCS), GTCS on awakening and myoclonic seizures. Defects in CLCN2 are associated with juvenile myoclonic epilepsy type 8 (EJM8). 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. Belongs to the chloride channel (TC 2.A.49) family. ClC-2/CLCN2 subfamily. 3 isoforms of the human protein are produced by alternative splicing.

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

Chromosomal Location of Human Ortholog: 3q27.1

Cellular Component: plasma membrane

Molecular Function: voltage-gated chloride channel activity

Biological Process: retina development in camera-type eye; transport; transmembrane transport

Disease: Epilepsy, Idiopathic Generalized, Susceptibility To, 11; Leukoencephalopathy With Ataxia

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