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RPGR Antibody (C-term)

Affinity Purified Rabbit Polyclonal Antibody (Pab)

     
  • WB - RPGR Antibody (C-term) AP12226b
    RPGR Antibody (C-term) (Cat. #AP12226b) western blot analysis in A549 cell line lysates (35ug/lane).This demonstrates the RPGR antibody detected the RPGR protein (arrow).
    detail
  • WB - RPGR Antibody (C-term) AP12226b
    Anti-RPGR Antibody (C-term) at 1:1000 dilution + human liver lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 113 kDa Blocking/Dilution buffer: 5% NFDM/TBST.
    detail
  • IHC-P - RPGR Antibody (C-term) AP12226b
    RPGR Antibody (C-term) (Cat. #AP12226b)immunohistochemistry analysis in formalin fixed and paraffin embedded human liver tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of RPGR Antibody (C-term) for immunohistochemistry. Clinical relevance has not been evaluated.
    detail
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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
IHC-P, WB, E
Primary Accession Q92834
Other Accession NP_000319.1
Reactivity Human
Host Rabbit
Clonality Polyclonal
Isotype Rabbit IgG
Calculated MW 113387 Da
Antigen Region 744-772 aa
Additional Information
Gene ID 6103
Other Names X-linked retinitis pigmentosa GTPase regulator, RPGR, RP3, XLRP3
Target/Specificity This RPGR antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 744-772 amino acids of human RPGR.
Dilution 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.
PrecautionsRPGR Antibody (C-term) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name RPGR
Synonyms RP3, XLRP3
Function Could be a guanine-nucleotide releasing factor. Plays a role in ciliogenesis. Probably regulates cilia formation by regulating actin stress filaments and cell contractility. Plays an important role in photoreceptor integrity. May play a critical role in spermatogenesis and in intraflagellar transport processes (By similarity). May be involved in microtubule organization and regulation of transport in primary cilia.
Cellular Location Cytoplasm, cytoskeleton, flagellum axoneme {ECO:0000250|UniProtKB:Q9R0X5}. Golgi apparatus. Cell projection, cilium {ECO:0000250|UniProtKB:Q9R0X5}. Note=In the retinal photoreceptor cell layer, localizes at the connecting cilium (By similarity). Colocalizes with WHRN in the photoreceptor connecting cilium (By similarity) Colocalizes with CEP290 in the photoreceptor connecting cilium (By similarity). Colocalizes with RPGRIP1 in the photoreceptor connecting cilium (By similarity). {ECO:0000250|UniProtKB:Q9R0X5}
Tissue Location Heart, brain, placenta, lung, liver, muscle, kidney, retina, pancreas and fetal retinal pigment epithelium. Isoform 3 is found only in the retina. Colocalizes with RPGRIP1 in the outer segment of rod photoreceptors and cone outer segments
Research Areas
Citations (0)
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Background

This gene encodes a protein with a series of six RCC1-like domains (RLDs), characteristic of the highly conserved guanine nucleotide exchange factors. The encoded protein is found in the Golgi body and interacts with RPGRIP1. This protein localizes to the outer segment of rod photoreceptors and is essential for their viability. Mutations in this gene have been associated with X-linked retinitis pigmentosa (XLRP). Multiple alternatively spliced transcript variants that encode different isoforms of this gene have been reported, but the full-length natures of only some have been determined.

References

Clark, G.R., et al. Ophthalmology 117(11):2169-2177(2010)
Schmid, F., et al. Invest. Ophthalmol. Vis. Sci. 51(3):1628-1635(2010)
Ji, Y., et al. Curr. Eye Res. 35(1):73-79(2010)
Sheng, X., et al. Mol. Vis. 16, 1620-1628 (2010) :
Murga-Zamalloa, C.A., et al. J. Genet. 88(4):399-407(2009)

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$ 365.00
$ 140.00
Cat# AP12226b
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