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

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Catalog # MBS7000090
Unit / Price
  0.05 mg  /  $160 +1 FREE 8GB USB
  0.1 mg  /  $235 +1 FREE 8GB USB
Product Name

RPGR, Polyclonal Antibody

Popular Item
Also Known As

RPGR Antibody

Product Synonym Names
X-linked retinitis pigmentosa GTPase regulator; RPGR; RP3; XLRP3
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence Length
815
OMIM
300029
3D Structure
ModBase 3D Structure for Q92834
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human
Purity/Purification
Affinity Purified
Form/Format
Liquid
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
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.
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

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
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
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
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
NCBI/Uniprot data below describe general gene information for RPGR. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
127,042 Da
NCBI Official Full Name
X-linked retinitis pigmentosa GTPase regulator isoform A
NCBI Official Synonym Full Names
retinitis pigmentosa GTPase regulator
NCBI Official Symbol
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
UniProt Gene Name
UniProt Synonym Gene Names
RP3; XLRP3  [Similar Products]
UniProt Entry Name
RPGR_HUMAN
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]
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
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.

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