CLC2, Rat Peptide | CLCN2 peptide
CLC2, Rat (Clcn2, Chloride Channel) Control Peptide
Highly purified
Highly purified
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.
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.
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
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.
Research Articles on CLCN2
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Product Notes
The CLCN2 clcn2 (Catalog #AAA658789) is a Peptide and is intended for research purposes only. The product is available for immediate purchase. AAA Biotech's CLC2, Rat can be used in a range of immunoassay formats including, but not limited to, 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. Researchers should empirically determine the suitability of the CLCN2 clcn2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "CLC2, Rat, Peptide" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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