Chloride Channel Protein 2 (CLCN2)

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CIC-2; CLC2; ECA3; EGI3; EGMA; EJM6

Chloride Channel Protein 2 (CLCN2)
CLC2 chloride channels are expressed in epithelia affected by cystic fibrosis (CF) and raised the possibility that these might represent an alternative target for pharmacotherapy of CF. The explore this possibility, they manipulated genetically the expression levels of CLC2 channels in airway epithelial cells derived from cystic fibrosis patients. Whole-cell patch-clamp analysis of cells overexpressing CLC2 identified hyperpolarization-activated chloride ion currents (HACCs) that displayed time- and voltage-dependent activation and an inwardly rectifying steady-state current voltage relationship. Reduction of extracellular pH to 5.0 caused significant increases in HACCs in overexpressing cells and the appearance of robust currents in parental cells from the cystic fibrosis patient.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Chloride Channel Protein 2 (CLCN2) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Chloride Channel Protein 2 (CLCN2) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Chloride Channel Protein 2 (CLCN2) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Chloride Channel Protein 2 (CLCN2) CLIA Kit Customized Service Offer
n/a ELISA Kit for Chloride Channel Protein 2 (CLCN2) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Chloride Channel Protein 2 (CLCN2) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Chloride Channel Protein 2 (CLCN2) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Chloride Channel Protein 2 (CLCN2) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Chloride Channel Protein 2 (CLCN2) CLIA Kit Customized Service Offer
n/a ELISA Kit for Chloride Channel Protein 2 (CLCN2) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Chloride Channel Protein 2 (CLCN2) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Chloride Channel Protein 2 (CLCN2) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Chloride Channel Protein 2 (CLCN2) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Chloride Channel Protein 2 (CLCN2) CLIA Kit Customized Service Offer
n/a ELISA Kit for Chloride Channel Protein 2 (CLCN2) ELISA Kit Customized Service Offer
  1. "Cloning of a putative human voltage-gated chloride channel (ClC-2) cDNA widely expressed in human tissues."Hum. Mol. Genet. 4:407-413(1995) [PubMed] [Europe PMC] [Abstract]
  2. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  3. "The DNA sequence, annotation and analysis of human chromosome 3." Nature 440:1194-1198(2006) [PubMed] [Europe PMC] [Abstract]
  4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  5. "Expression of CLCN voltage-gated chloride channel genes in human blood vessels."J. Mol. Cell. Cardiol. 31:657-666(1999) [PubMed] [Europe PMC] [Abstract]
  6. "Voltage-dependent and -independent titration of specific residues accounts for complex gating of a ClC chloride channel by extracellular protons."J. Physiol. (Lond.) 587:1387-1400(2009) [PubMed] [Europe PMC] [Abstract]
  7. "N-terminal acetylome analyses and functional insights of the N-terminal acetyltransferase NatB."Proc. Natl. Acad. Sci. U.S.A. 109:12449-12454(2012) [PubMed] [Europe PMC] [Abstract]
  8. "Mutations in CLCN2 encoding a voltage-gated chloride channel are associated with idiopathic generalized epilepsies." Nat. Genet. 33:527-532(2003) [PubMed] [Europe PMC] [Abstract]
  9. "Alterations in the cytoplasmic domain of CLCN2 result in altered gating kinetics."Cell. Physiol. Biochem. 20:441-454(2007) [PubMed] [Europe PMC] [Abstract]
  10. "Clinical and genetic familial study of a large cohort of Italian children with idiopathic epilepsy."Brain Res. Bull. 79:89-96(2009) [PubMed] [Europe PMC] [Abstract]
  11. "Two novel CLCN2 mutations accelerating chloride channel deactivation are associated with idiopathic generalized epilepsy."Hum. Mutat. 30:397-405(2009) [PubMed] [Europe PMC] [Abstract]
  12. "Brain white matter oedema due to ClC-2 chloride channel deficiency: an observational analytical study." Lancet Neurol. 12:659-668(2013) [PubMed] [Europe PMC] [Abstract]