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anti-RPGR antibody :: Rabbit anti-Human RPGR Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS7000090
Unit / Price
0.05 mg  /  $160 +1 FREE 8GB USB
0.1 mg  /  $235 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

RPGR, Polyclonal Antibody

★Popular Item★
 Also Known As   

RPGR Antibody

 Product Synonym Names    X-linked retinitis pigmentosa GTPase regulator; RPGR; RP3; XLRP3
 Product Gene Name   

anti-RPGR antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    300029
 3D Structure    ModBase 3D Structure for Q92834
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human
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 Purity/Purification    Affinity Purified
 Form/Format    Liquid
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 Immunogen    Recombinant human X-linked retinitis pigmentosa GTPase regulator protein
 Conjugation    Non-conjugated
 Storage Buffer    Preservative: 0.03% Proclin 300; Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
 Santa Cruz Alternative    Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-14672
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 Preparation and Storage    Shipped at 4 degree C. Upon delivery, aliquot and store at -20 degree C or -80 degree C.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Other Notes    Small volumes of anti-RPGR antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for anti-RPGR antibody

   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.
 Applications Tested/Suitable for anti-RPGR antibody   

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

 Application Notes for anti-RPGR antibody    Recommended dilution: WB:1:500-2000, IHC:1:20-1:200
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 Western Blot (WB) of anti-RPGR antibody    Western blot
All lanes: RPGR antibody at 6ug/ml
Lane 1: 293T whole cell lysate
Lane 2: Hela whole cell lysate
Secondary
Goat polyclonal to Rabbit IgG at 1/10000 dilution
Predicted band size: 114,91,71,84,53,128 kDa
Observed band size: 113 kDa

anti-RPGR antibody Western Blot (WB) (WB) image
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 Immunohistochemistry (IHC) of anti-RPGR antibody    Immunohistochemistry of paraffin-embedded human kidney tissue using MBS7000090 at dilution of 1:100
anti-RPGR antibody Immunohistochemistry (IHC) (IHC) image
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 Immunohistochemistry (IHC) of anti-RPGR antibody    Immunohistochemistry of paraffin-embedded human liver cancer using MBS7000090 at dilution of 1:100
anti-RPGR antibody Immunohistochemistry (IHC) (IHC) image
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 Immunofluorescence (IF) of anti-RPGR antibody    Immunofluorescent analysis of PC3 cells cells using MBS7000090 at a dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
anti-RPGR antibody Immunofluorescence (IF) image
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NCBI/Uniprot data below describe general gene information for RPGR. It may not necessarily be applicable to this product.
 NCBI GI #    4506581
 NCBI GeneID    6103
 NCBI Accession #    NP_000319.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_000328.2 [Other Products]
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 UniProt Primary Accession #    Q92834 [Other Products]
 UniProt Secondary Accession #    O00702; O00737; Q3KN84; Q8N5T6; Q93039; Q9HD29; Q9UMR1; B1ARN3; E9PE28 [Other Products]
 UniProt Related Accession #    Q92834 [Other Products]
 Molecular Weight    127,042 Da
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 NCBI Official Full Name    X-linked retinitis pigmentosa GTPase regulator isoform A
 NCBI Official Synonym Full Names    retinitis pigmentosa GTPase regulator
 NCBI Official Symbol    RPGR [Similar Products]
 NCBI Official Synonym Symbols   
CRD; RP3; COD1; PCDX; RP15; XLRP3; orf15; CORDX1
[Similar Products]
 NCBI Protein Information    X-linked retinitis pigmentosa GTPase regulator
 UniProt Protein Name    X-linked retinitis pigmentosa GTPase regulator
 Protein Family    X-linked retinitis pigmentosa GTPase regulator
 UniProt Gene Name    RPGR [Similar Products]
 UniProt Synonym Gene Names    RP3; XLRP3 [Similar Products]
 UniProt Entry Name    RPGR_HUMAN
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 NCBI Summary for RPGR    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. [provided by RefSeq, Dec 2008]
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 UniProt Comments for RPGR    RPGR: a guanine nucleotide exchange factor (GEF) that plays a role in cilia biogenesis and maintenance. Interacts with the GDP-bound form of the small GTPase RAB8A. Promotes the exchange of GDP to GTP, converting inactive GDP-bound Rab proteins into their active GTP-bound form. 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 May be involved in microtubule organization and regulation of transport in primary cilia. Interacts with PDE6D, RPGRIP1, CEP290, and DFNB31. Defects in RPGR are the cause of retinitis pigmentosa (RP3, RP15, and RPDSI), cone-rod dystrophy, X-linked 1 (CORDX1), and macular degeneration, X-linked, atrophic (MDXLA). Six isoforms of the human protein are produced by alternative splicing. Isoform 3 is found only in the retina. Colocalizes with RPGRIP1 in the outer segment of rod photoreceptors and cone outer segments. Isoform 6 associates with microtubule organizing centers, cilium basal bodies, and cilium axonemes. Interacts with NPM1 (via C-terminus), RPGRIP1L, SMC1A and SMC3.

Protein type: GEFs; GEFs, Rab

Chromosomal Location of Human Ortholog: Xp21.1

Cellular Component: centrosome; Golgi apparatus; photoreceptor connecting cilium; photoreceptor outer segment

Molecular Function: guanyl-nucleotide exchange factor activity; protein binding

Biological Process: cilium biogenesis; eye photoreceptor cell development; intracellular protein transport; intraflagellar transport; positive regulation of GTPase activity; retina morphogenesis in camera-type eye; visual perception

Disease: Cone-rod Dystrophy, X-linked, 1; Macular Degeneration, X-linked Atrophic; Retinitis Pigmentosa 3; Retinitis Pigmentosa, X-linked, And Sinorespiratory Infections, With Or Without Deafness
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 Research Articles on RPGR    1. The regulator of chromosome condensation 1-like domain of RPGR was conserved in vertebrates and invertebrates, but RPGR(ORF15) was unique to vertebrates.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-RPGR antibodyDiseases associated with anti-RPGR antibody
 Reference Product  PubMed Publications
 RPGRIP1 antibody  >35 publications with RPGR and RPGRIP1
 PDE6D antibody  >4 publications with RPGR and PDE6D
 IQCB1 antibody  >3 publications with RPGR and IQCB1
 RANBP2 antibody  >2 publications with RPGR and RANBP2
 SMC3 antibody  >2 publications with RPGR and SMC3
 IFT88 antibody  >2 publications with RPGR and IFT88
 KIF3A antibody  >1 publications with RPGR and KIF3A
 Disease Name  Pubmed Publications
 Retinitis Pigmentosa, X-Linked, And Sinorespiratory Infections, With Or Without Deafness Antibodies  >35687 publications with RPGR and Retinitis Pigmentosa, X-Linked, And Sinorespiratory Infections, With Or Without Deafness
 Retinitis Pigmentosa Antibodies  >257 publications with RPGR and Retinitis Pigmentosa
 Retinal Degeneration Antibodies  >228 publications with RPGR and Retinal Degeneration
 Retinal Dystrophies Antibodies  >198 publications with RPGR and Retinal Dystrophies
 CONE-ROD DYSTROPHY, X-LINKED, 1 Antibodies  >91 publications with RPGR and CONE-ROD DYSTROPHY, X-LINKED, 1
 Retinitis Pigmentosa 3 Antibodies  >77 publications with RPGR and Retinitis Pigmentosa 3
 Atrophy Antibodies  >18 publications with RPGR and Atrophy
 Macular Degeneration Antibodies  >12 publications with RPGR and Macular Degeneration
 Hearing Loss Antibodies  >12 publications with RPGR and Hearing Loss
 Chromosome Aberrations Antibodies  >7 publications with RPGR and Chromosome Aberrations
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