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anti-Chloride Channel, Voltage-Sensitive 7 (CLCN7) Antibodies

The product of CLCN7 belongs to the CLC chloride channel family of proteins. Additionally we are shipping Chloride Channel, Voltage-Sensitive 7 Proteins (7) and many more products for this protein.

list all antibodies Gene Name GeneID UniProt
CLCN7 26373 O70496
CLCN7 1186 P51798
CLCN7 29233 P51799
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Top anti-Chloride Channel, Voltage-Sensitive 7 Antibodies at antibodies-online.com

Showing 10 out of 54 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Supplier Delivery Price Details
Human Rabbit Un-conjugated EIA, IF, IHC (p), WB Confocal immunofluorescent analysis of CLCN7 Antibody (C-term)(Cat#AP50940PU-N) with WiDr cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue). CLCN7 Antibody (C-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human kidney tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of CLCN7 Antibody (C-term) for immunohistochemistry. Clinical relevance has not been evaluated. 0.4 mL Log in to see 6 to 8 Days
$401.50
Details
Human Rabbit Un-conjugated ELISA, WB   100 μg Log in to see 2 to 3 Days
$302.50
Details
Human Rabbit Un-conjugated IF, IHC (p), WB CLCN7 Antibody (C-term)  western blot analysis in CEM cell line lysates (35ug/lane).This demonstrates the CLCN7 antibody detected the CLCN7 protein (arrow). CLCN7 Antibody (C-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human kidney tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of CLCN7 Antibody (C-term) for immunohistochemistry.  Clinical relevance has not been evaluated. 400 μL Log in to see 10 to 11 Days
$324.50
Details
Human Rabbit Un-conjugated IC, IF, WB Immunofluorescent analysis of CLCN7 staining in HeLa cells. Formalin-fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 5-10 minutes and blocked with 3% BSA-PBS for 30 minutes at room temperature. Cells were probed with the primary antibody in 3% BSA-PBS and incubated overnight at 4 °C in a humidified chamber. Cells were washed with PBST and incubated with a DyLight 594-conjugated secondary antibody (red) in PBS at room temperature in the dark. DAPI was used to stain the cell nuclei (blue). Western blot analysis of CLCN7 expression in HeLa (A), mouse liver (B), rat liver (C) whole cell lysates. 200 μL Log in to see 9 Days
$487.50
Details
Rat Rabbit Un-conjugated ELISA, WB   50 μL Log in to see 8 to 10 Days
$566.50
Details
Human Rabbit Un-conjugated ELISA, WB   50 μL Log in to see 8 to 10 Days
$566.50
Details
Human Mouse Un-conjugated ELISA, WB Western Blot detection against Immunogen (36.74 KDa) . 200 μL Log in to see 9 Days
$430.00
Details
Human Rabbit Un-conjugated IF, IHC, WB 400 μL Log in to see 6 Days
$457.14
Details
Human Rabbit Un-conjugated IF, IHC, ELISA, WB   50 μg Log in to see 8 to 10 Days
$610.50
Details
Human Rabbit PE ICC, IHC, ELISA, WB   200 μL Log in to see 8 to 10 Days
$969.83
Details

CLCN7 Antibodies by Reactivity, Application, Clonality and Conjugate

Attributes Applications Host Clonality Conjugate
Mouse (Murine) ,


Human , , , ,
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Top referenced anti-Chloride Channel, Voltage-Sensitive 7 Antibodies

  1. Human Polyclonal CLCN7 Primary Antibody for EIA, IF - ABIN951556 : Furthner, Biebl, Weinzettel, Schmitt, Lahr, Ebetsberger, Rittinger, Schulz: Osteopetrosis due to homozygous chloride channel ClCN7 mutation mimicking metabolic disease with haematological and neurological impairment. in Klinische Pädiatrie 2010 (PubMed)
    Show all 3 references for ABIN951556

  2. Human Polyclonal CLCN7 Primary Antibody for ELISA, WB - ABIN1451157 : Martin, Han, Gordon, Terry, Prabhakar, She, Xie, Hellsten, Chan, Altherr, Couronne, Aerts, Bajorek, Black, Blumer, Branscomb, Brown, Bruno, Buckingham, Callen, Campbell, Campbell, Campbell, Caoile et al.: The sequence and analysis of duplication-rich human chromosome 16. ... in Nature 2004 (PubMed)

More Antibodies against Chloride Channel, Voltage-Sensitive 7 Interaction Partners

Mouse (Murine) Chloride Channel, Voltage-Sensitive 7 (CLCN7) interaction partners

  1. ClC-7 is essential for osteoclasts to resorb craniofacial bones to enable tooth eruption and root development.

  2. ClC-7 deficiency impacts the development of the dentition and calvaria, but does not significantly disrupt amelogenesis.

  3. ClC-7 does not appear to be crucially involved in gastric acid secretion, which explains the absence of an osteopetrorickets phenotype in CLCN7-related osteopetrosis (show CSF1 Antibodies).

  4. Findings in this knockout mouse model prove that osteopetrotic compression of the brain is not responsible for neuronal and retinal degeneration in CLCN7-deficient mice; rather, they suggest that neurotoxicity is most likely due to lysosomal dysfunction.

  5. Degradation of Alzheimer's amyloid fibrils by microglia requires delivery of chloride channel 7 to lysosomes. [ClC7]

  6. Chloride channel 7 (Clcn7) deficient mice bear a close resemblance to the progressive neuropathologic phenotype of neuronal ceroid lipofuscinosis.

  7. study of mice with a point mutation converting ClC-7 into an uncoupled (unc) Cl-conductor; findings show only some roles of ClC-7 Cl-/H+ exchange can be taken over by a Cl- conductance

  8. These experiments demonstrate that lysosomal pathology is a cell-autonomous consequence of ClC-7 disruption and that ClC-7 is important for lysosomal protein degradation.

  9. ClC-7 knockout mice display neurodegeneration and severe lysosomal storage disease despite unchanged lysosomal pH in cultured neurons.

  10. both ClC-7 and Ostm1 (show OSTM1 Antibodies) proteins co-localize in late endosomes and lysosomes of various tissues, as well as in the ruffled border of bone-resorbing osteoclasts

Cow (Bovine) Chloride Channel, Voltage-Sensitive 7 (CLCN7) interaction partners

  1. accelerated ClC-7/Ostm1 (show OSTM1 Antibodies) gating per se is deleterious, highlighting a physiological importance of the slow voltage-activation of ClC-7/Ostm1 (show OSTM1 Antibodies) in lysosomal function and bone resorption

Human Chloride Channel, Voltage-Sensitive 7 (CLCN7) interaction partners

  1. study demonstrates a wide heterogeneity in the progression of the phenotypes and expanded the mutational spectrum for the CLCN7 gene

  2. the unusual clinical presentation observed in our patient with a mild clinical onset evolving towards a more serious clinical picture, is associated to two novel mutations on CLCN7 gene.

  3. Results show that ClC-7 is strongly expressed in OUMS-27,a chondrocyte cell line and is responsible for Cl- current. Its downregulation during the hypoosmotic stress accompanying osteoarthritis progression is part of the complex etiology of the disease.

  4. analysis demonstrates that CLCN7 and TCIRG1 (show TCIRG1 Antibodies) mutations differentially affect bone matrix mineralization, and that there is a need to modify the current classification of osteopetrosis (show CSF1 Antibodies)

  5. recurrent p.Gly215Arg mutation and novel missense mutations p.Ala299Val and p.Trp319Arg in the CLCN7 gene were responsible for these three Chinese ADO (show ADO Antibodies)-II families.

  6. ClC-7 does not appear to be crucially involved in gastric acid secretion, which explains the absence of an osteopetrorickets phenotype in CLCN7-related osteopetrosis (show CSF1 Antibodies).

  7. Eight mutations, including two reported mutations (R767W and E798FS) and six novel mutations (E313K, A316G, R743W, G741R, W127G and S290F), were detected in the CLCN7 gene from 12 living autosomal dominant osteopetrosis (show CSF1 Antibodies) type II patients.

  8. The authors show that both the aminoterminus and transmembrane span of the Ostm1 (show OSTM1 Antibodies) beta-subunit (show POLG Antibodies) are required for ClC-7 Cl(-)/H(+)-exchange, whereas the Ostm1 (show OSTM1 Antibodies) transmembrane domain suffices for its ClC-7-dependent trafficking to lysosomes.

  9. Rat G213R ClC-7 is the analogue of human G215R ClC-7 responsible for autosomal dominant osteopetrosis (show CSF1 Antibodies) type II.

  10. Elevated serum LDH isoenzymes and AST indicate a disturbance (of uncertain clinical significance) within multiple extraosseous tissues when there is CLCN7 deficiency.

Chloride Channel, Voltage-Sensitive 7 (CLCN7) Antigen Profile

Protein Summary

The product of this gene belongs to the CLC chloride channel family of proteins. Chloride channels play important roles in the plasma membrane and in intracellular organelles. This gene encodes chloride channel 7. Defects in this gene are the cause of osteopetrosis autosomal recessive type 4 (OPTB4), also called infantile malignant osteopetrosis type 2 as well as the cause of autosomal dominant osteopetrosis type 2 (OPTA2), also called autosomal dominant Albers-Schonberg disease or marble disease autosoml dominant. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. OPTA2 is the most common form of osteopetrosis, occurring in adolescence or adulthood.

Gene names and symbols associated with CLCN7

  • chloride channel 7 (Clcn7) antibody
  • chloride channel 7 (CLCN7) antibody
  • chloride channel, voltage-sensitive 7 (CLCN7) antibody
  • chloride channel, voltage-sensitive 7 (Clcn7) antibody
  • AA409691 antibody
  • AW538136 antibody
  • CLC-7 antibody
  • CLC7 antibody
  • D17Wsu51e antibody
  • OPTA2 antibody
  • OPTB4 antibody
  • PPP1R63 antibody

Protein level used designations for CLCN7

H(+)/Cl(-) exchange transporter 7 , chloride channel 7 alpha subunit , chloride channel protein 7 , protein phosphatase 1, regulatory subunit 63

GENE ID SPECIES
26373 Mus musculus
513545 Bos taurus
1186 Homo sapiens
29233 Rattus norvegicus
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