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CLCN7 Antibody (C-term)

Affinity Purified Rabbit Polyclonal Antibody (Pab)

     
  • IF - CLCN7 Antibody (C-term) AP11863B
    Confocal immunofluorescent analysis of CLCN7 Antibody (C-term)(Cat#AP11863b) with WiDr cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).
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  • WB - CLCN7 Antibody (C-term) AP11863B
    CLCN7 Antibody (C-term) (Cat. #AP11863b) western blot analysis in CEM cell line lysates (35ug/lane).This demonstrates the CLCN7 antibody detected the CLCN7 protein (arrow).
    detail
  • IHC-P - CLCN7 Antibody (C-term) AP11863B
    CLCN7 Antibody (C-term) (Cat. #AP11863b)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.
    detail
  • SPECIFICATION
  • CITATIONS: 1
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  • BACKGROUND
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Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
WB, IF, IHC-P, E
Primary Accession P51798
Other Accession P51799, O70496, Q4PKH3, NP_001278.1
Reactivity Human
Predicted Bovine, Mouse, Rat
Host Rabbit
Clonality Polyclonal
Isotype Rabbit IgG
Calculated MW 88679 Da
Antigen Region 692-720 aa
Additional Information
Gene ID 1186
Other Names H(+)/Cl(-) exchange transporter 7, Chloride channel 7 alpha subunit, Chloride channel protein 7, ClC-7, CLCN7
Target/Specificity This CLCN7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 692-720 amino acids from the C-terminal region of human CLCN7.
Dilution IF~~1:10~50
WB~~1:1000
IHC-P~~1:10~50
Format Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
StorageMaintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsCLCN7 Antibody (C-term) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name CLCN7 (HGNC:2025)
Function Slowly voltage-gated channel mediating the exchange of chloride ions against protons (PubMed:18449189, PubMed:21527911). Functions as antiporter and contributes to the acidification of the lysosome lumen and may be involved in maintaining lysosomal pH (PubMed:18449189, PubMed:21527911, PubMed:31155284). The CLC channel family contains both chloride channels and proton-coupled anion transporters that exchange chloride or another anion for protons (By similarity). The presence of conserved gating glutamate residues is typical for family members that function as antiporters (By similarity).
Cellular Location Lysosome membrane; Multi-pass membrane protein
Tissue Location Brain and kidney..
Research Areas
Citations ( 0 )

Background

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.

References

Furthner, D., et al. Klin Padiatr 222(3):180-183(2010)
Phadke, S.R., et al. Indian J. Med. Res. 131, 508-514 (2010) :
Pangrazio, A., et al. Hum. Mutat. 31 (1), E1071-E1080 (2010) :
Kajiya, H., et al. Pflugers Arch. 458(6):1049-1059(2009)
Mazzolari, E., et al. Am. J. Hematol. 84(8):473-479(2009)

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$ 365.00
$ 140.00
Cat# AP11863B
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