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

The product of CLCN7 belongs to the CLC chloride channel family of proteins. De plus, nous expédions Chloride Channel, Voltage-Sensitive 7 Protéines (7) et beaucoup plus de produits pour cette protéine.

afficher tous les anticorps Gène GeneID UniProt
CLCN7 1186 P51798
CLCN7 26373 O70496
CLCN7 29233 P51799
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Top anti-Chloride Channel, Voltage-Sensitive 7 Anticorps sur anticorps-enligne.fr

Showing 10 out of 56 products:

Catalogue No. Reactivité Hôte Conjugué Application Images Quantité Fournisseur Livraison Prix Détails
Humain Lapin Inconjugué 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 Connectez-vous pour afficher 13 to 14 Days
$487.50
Détails
Humain Lapin Inconjugué 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 Connectez-vous pour afficher 10 to 11 Days
$335.50
Détails
Humain Lapin Inconjugué ELISA, WB 100 μL Connectez-vous pour afficher 16 Days
$181.73
Détails
Humain Lapin Inconjugué EIA, IF, IHC (p), WB   0.4 mL Connectez-vous pour afficher 8 to 11 Days
$517.00
Détails
Humain Lapin Inconjugué ELISA, ICC, IF, WB Western blot analysis of extracts from A549 cells using CLCN7 antibody.,The lane on the left is treated with the antigen-specific peptide. ABIN6275003 staining A549 by IF/ICC. The sample were fixed with PFA and permeabilized in 0.1% Triton X-100,then blocked in 10% serum for 45 minutes at 25¡ãC. The primary antibody was diluted at 1/200 and incubated with the sample for 1 hour at 37¡ãC. An  Alexa Fluor 594 conjugated goat anti-rabbit IgG (H+L) Ab, diluted at 1/600, was used as the secondary antibod 100 μL Connectez-vous pour afficher 11 to 12 Days
$390.77
Détails
Humain Lapin Inconjugué ELISA, WB   100 μg Connectez-vous pour afficher 2 to 3 Days
$302.50
Détails
Humain Lapin Inconjugué ELISA, WB 200 μL Connectez-vous pour afficher 12 to 14 Days
$438.90
Détails
Humain Lapin Inconjugué IHC, WB Western blot analysis of extracts of various cell lines, using CLCN7 antibody (ABIN5974147) at 1/1000 dilution. Immunohistochemistry of paraffin-embedded mouse brain using CLCN7 antibody (ABIN5974147) at dilution of 1/100 (40x lens). 100 μL Connectez-vous pour afficher 11 to 16 Days
$426.40
Détails
Humain Lapin Inconjugué ELISA, WB   100 μg Connectez-vous pour afficher 2 to 3 Days
$302.50
Détails
Humain Souris Inconjugué ELISA, WB Western Blot detection against Immunogen (36.74 KDa) . 200 μL Connectez-vous pour afficher 11 to 12 Days
$354.20
Détails

anti-Chloride Channel, Voltage-Sensitive 7 Anticorps mieux référencés

  1. Human Polyclonal CLCN7 Primary Antibody pour IF, IHC (p) - ABIN655808 : Yoshimoto, Morita, Matsubara, Mitsuyasu, Imai, Kajioka, Yoneda, Ito, Hirofuji, Nakamura, Hirata: Surface vacuolar ATPase in ameloblastoma contributes to tumor invasion of the jaw bone. dans International journal of oncology 2016 (PubMed)

Plus d’anticorps contre Chloride Channel, Voltage-Sensitive 7 partenaires d’interaction

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

  1. In this study, whole exome sequencing (WES) was successfully used in six patients with malignant infantile osteopetrosis (Montrer CSF1 Anticorps) (MIOP) and identified mutations in four MIOP-related genes (CLCN7, TCIRG1 (Montrer TCIRG1 Anticorps), SNX10 (Montrer SNX10 Anticorps), and TNFRSF11A (Montrer TNFRSF11A Anticorps)).

  2. The present study revealed three novel mutations, showed the dense but brittle sclerotic bones of an autosomal dominant osteopetrosis (Montrer CSF1 Anticorps) type II (OPTA2) patient, characterized OPTA2 symptoms from benign to fatal and reported a rare intermediate case of autosomal recessive 4 in a Chinese population.

  3. we identified a CLCN7 mutation in a family with autosomal dominant osteopetrosis (Montrer CSF1 Anticorps), RTA (Montrer RBM9 Anticorps), renal stones, epilepsy, and blindness.

  4. present findings suggest that the novel missense mutations V289L and A542V in the CLCN7 gene were responsible for autosomal dominant osteopetrosis (Montrer CSF1 Anticorps) (type II) in the two Chinese families.

  5. Exome sequencing and Sanger sequencing were conducted in Han Chinese family members, some of whom had typical osteopetrosis, and a novel missense variant c.2350A>T (p.R784W) in the chloride channel 7 gene (CLCN7) was identified.

  6. The present study identified seven novel mutations of the CLCN7 gene and reported the first case of intermediate autosomal recessive osteopetrosis (Montrer CSF1 Anticorps). with compound heterozygous mutation in the Chinese population.

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

  8. 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.

  9. 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.

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

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

  1. a mouse with a different osteopetrosis-causing mutation (Clcn7(F318L)), is defined.

  2. ClC-7 may affect tooth development by directly targeting tooth cells, and regulate tooth eruption through DFC mediated osteoclast pathway.

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

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

  5. 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 (Montrer CSF1 Anticorps).

  6. 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.

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

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

  9. 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

  10. 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.

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

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

Chloride Channel, Voltage-Sensitive 7 (CLCN7) profil antigène

Profil protéine

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 voltage-gated channel 7 (CLCN7) anticorps
  • chloride channel, voltage-sensitive 7 (Clcn7) anticorps
  • chloride voltage-gated channel 7 (Clcn7) anticorps
  • chloride channel, voltage-sensitive 7 L homeolog (clcn7.L) anticorps
  • chloride channel 7 (clcn7) anticorps
  • chloride channel, voltage-sensitive 7 (clcn7) anticorps
  • chloride channel protein 7 (CpipJ_CPIJ008618) anticorps
  • AA409691 anticorps
  • AW538136 anticorps
  • ClC-7 anticorps
  • CLC7 anticorps
  • CLCN7 anticorps
  • D17Wsu51e anticorps
  • im:7155923 anticorps
  • MGC80627 anticorps
  • OPTA2 anticorps
  • OPTB4 anticorps
  • PPP1R63 anticorps
  • zgc:153490 anticorps

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 , chloride channel 7 , H(+)/Cl(-) exchange transporter 7-like

GENE ID SPECIES
1186 Homo sapiens
26373 Mus musculus
29233 Rattus norvegicus
416656 Gallus gallus
444161 Xenopus laevis
513545 Bos taurus
553351 Danio rerio
733969 Xenopus (Silurana) tropicalis
6041049 Culex quinquefasciatus
100429296 Macaca mulatta
453817 Pan troglodytes
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