CLCN2 Antibody

Code CSB-PA005481LA01HU
Size US$166
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  • Immunofluorescent analysis of HepG2 cells using CSB-PA005481LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) CLCN2 Polyclonal antibody
Uniprot No.
Target Names
CLCN2
Alternative Names
Chloride Channel 2 antibody; Chloride channel protein 2 antibody; Chloride channel; voltage sensitive 2 antibody; CIC 2 antibody; CIC2 antibody; ClC-2 antibody; CLC2 antibody; Clcn2 antibody; CLCN2_HUMAN antibody; ECA2 antibody; ECA3 antibody; EG13 antibody; EGI11 antibody; EGMA antibody; EJM6 antibody; EJM8 antibody; PKA-activated chloride channel antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Chloride channel protein 2 protein (549-770AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The CLCN2 Antibody (Product code: CSB-PA005481LA01HU) is Non-conjugated. For CLCN2 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA005481LB01HU CLCN2 Antibody, HRP conjugated ELISA
FITC CSB-PA005481LC01HU CLCN2 Antibody, FITC conjugated
Biotin CSB-PA005481LD01HU CLCN2 Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form
Liquid
Tested Applications
ELISA, IF
Recommended Dilution
Application Recommended Dilution
IF 1:50-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume, membrane potential stabilization, signal transduction and transepithelial transport. Involved in the regulation of aldosterone production. The opening of CLCN2 channels at hyperpolarized membrane potentials in the glomerulosa causes cell membrane depolarization, activation of voltage-gated Ca2+ channels and increased expression of aldosterone synthase, the rate-limiting enzyme for aldosterone biosynthesis.
Gene References into Functions
  1. The inhibitory effects of ClC-2 knockout on HBVSMC proliferation and motility were associated with inactivation of the Wnt/beta-catenin signaling pathway, as evidenced by inhibition of beta-catenin phosphorylation and nuclear translocation, and decrease of GSK-3beta phosphorylation and survivin and cyclin D1 expression. PMID: 29988306
  2. Leukoencephalopathy-causing CLCN2 mutations impair chloride channel gating and trafficking. PMID: 28905383
  3. ClC-2 mRNA level was significantly increased in patients with non-alcoholic steatohepatitis, which positively correlated with the plasma levels of alanine transaminase (ALT), aspartate transaminase (AST) and insulin. PMID: 29550812
  4. The findings show that RGET691 of human ClC-2 (possible binding site) plays an important functional role in fatty acid activation of human ClC-2. PMID: 28424169
  5. Hyperpolarization activates CLC-2 mainly by driving intracellular anions into the channel pores. PMID: 26666914
  6. The extracellular domain of GlialCAM is necessary for cell junction targeting and for mediating interactions with itself or with MLC1 and ClC-2. PMID: 26033718
  7. both ubiquitous (AP-1A) and epithelium-specific (AP-1B) forms of the tetrameric clathrin adaptor AP-1 are capable of carrying out basolateral sorting of ClC-2. PMID: 25739457
  8. SPAK and OSR1 are powerful negative regulators of the cell volume regulatory Cl- channel ClC-2 PMID: 25323061
  9. Correlation between CLC-2 gene expression and the cytoskeleton in human trabecular meshwork cells. PMID: 23934342
  10. Our observations substantiate the concept that ClC-2 is involved in brain ion and water homoeostasis PMID: 23707145
  11. JAK2 down-regulates ClC-2 activity and thus counteracts Cl(-) exit, an effect which may impact on cell volume regulation PMID: 22613974
  12. This study demonistrated that the first auxiliary subunit of ClC-2 and suggests that ClC-2 may play a role in the pathology of MLC disease. PMID: 22405205
  13. ClC-2 plays an important role in the modulation of tight junctions by influencing caveolar trafficking of tight junction protein occludin. PMID: 21956164
  14. Sequence analysis of CLCN2 at genomic DNA and cDNA levels in 18 megalencephalic leukoencephalopathy MLC1 mutations revealed some nucleotide changes, but they were predicted to be nonpathogenic. PMID: 20187760
  15. found significant expression of ClC-2 at the apex of ciliated cells in both rat and human airways PMID: 11880269
  16. a functional interaction between the ClC-2 chloride channel and the retrograde motor dynein complex. PMID: 12601004
  17. ClC-2 and ClC-3 channels are specifically upregulated in glioma membranes and endow glioma cells with an enhanced ability to transport Chloride PMID: 12843258
  18. activation of hClC-2 is differentially regulated by PKA at two sites PMID: 15010473
  19. Functional evaluation of mutated channels associated with idiopathic generalized epilepsies PMID: 15252188
  20. activation of host ClC-2 channels participates in the altered permeability of Plasmodium-infected erythrocytes but is not required for intraerythrocytic parasite survival PMID: 15272009
  21. These results indicate that ClC-2 may not be a Cl- -transporting protein for gastric acid secretion in parietal cells. PMID: 15388342
  22. These data suggest that interferon-gamma activates ClC-2 channel activity in lung epithelial cells via mRNA stabilization. PMID: 15464978
  23. The authors conclude that CLCN2 mutations may be a rare cause of familial epilepsy. Further studies are needed to test if polymorphisms in this gene are associated with epilepsy. PMID: 15505175
  24. CLC-2 is not a key modifier gene of Cystic fibrosis lung disease phenotype. PMID: 15507145
  25. CLCN2 is related to idiopathic generalized epilepsy. PMID: 15508929
  26. association of Hsp90 with ClC-2 results in greater channel activity due to increased cell surface channel expression, facilitation of channel opening, and enhanced channel sensitivity to intracellular [Cl-] PMID: 16049054
  27. This suggests that slow and fast gating in ClC-2 are coupled, perhaps with slow gating contributing to the operation of the pore E207 as a protopore gate. PMID: 16469788
  28. mutations in the CLCN2 gene are only a rare cause of idiopathic generalized epilepsy PMID: 16932951
  29. CLCN2 may be a susceptibility locus in a subset of cases of childhood absence epilepsy PMID: 17580110
  30. Africans' gene pool comprises CLCN2 gene variants in the N-terminus, the C-terminus or the pore domain that affect surface expression and voltage- or cell-swelling-stimulated channel gating PMID: 17762171
  31. CLC-2 is upregulated in ethmoid mucosa and may affect the development of chronic rhinosinusitis without nasal polyps. PMID: 17882904
  32. propose that the function of the ClC-2 carboxy-terminus is to slow down the time course of channel activation in order to stabilize neuronal excitability PMID: 18801843
  33. Finding predicts a loss of function that may contribute to intracellular chloride accumulation or neuronal hyperexcitability. PMID: 19191339
  34. PIKfyve is a potent stimulator of ClC-2-activity and contributes to SGK1-dependent regulation of ClC-2. PMID: 19232516
  35. GaTx2 specifically inhibits ClC-2 channels, showing no inhibitory effect on a battery of other major classes of chloride channels and voltage-gated potassium channels. GaTx2 is the first peptide toxin inhibitor of any ClC protein. PMID: 19574231
  36. CLCN2 variants in indiopathic generalized epilepsy are reported. PMID: 19710712
  37. ClC-2 requires residing in beta-cyclodextrin sensitive plasma membrane clusters with other molecules in order to remain active. Regulation of ClC-2 trafficking to (endocytic pathway) and within the membrane could be a means of modulating its activity. PMID: 19711355
  38. two novel CLCN2 missense mutations, p.Arg235Gln and p.Arg577Gln, accelerating chloride channel deactivation are associated with idiopathic generalized epilepsy. PMID: 19191339

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Involvement in disease
Epilepsy, idiopathic generalized 11 (EIG11); Juvenile absence epilepsy 2 (JAE2); Juvenile myoclonic epilepsy 8 (EJM8); Leukoencephalopathy with ataxia (LKPAT)
Subcellular Location
Cell membrane; Multi-pass membrane protein.
Protein Families
Chloride channel (TC 2.A.49) family, ClC-2/CLCN2 subfamily
Tissue Specificity
Ubiquitously expressed. Moderately expressed in aortic and coronary vascular smooth muscle cells and expressed at a low level in aortic endothelial cells. Expressed in the adrenal gland, predominantly in the zona glomerulosa.
Database Links

HGNC: 2020

OMIM: 600570

KEGG: hsa:1181

STRING: 9606.ENSP00000265593

UniGene: Hs.436847

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