Full Product Name
RPGR Rabbit Polyclonal
Product Gene Name
anti-RPGR antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse
Purity/Purification
>=95% as determined by SDS-PAGE
Immunogen Affinity Purified
Immunogen
Retinitis pigmentosa GTPase regulator
Preparation and Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 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.
Applications Tested/Suitable for anti-RPGR antibody
ELISA (EIA), Western Blot (WB), Immunofluorescence (IF), Immunohistochemistry (IHC)
NCBI/Uniprot data below describe general gene information for RPGR. It may not necessarily be applicable to this product.
NCBI Accession #
AKI72611.1
[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
NCBI Official Full Name
RPGR, partial
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
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, Rab; GEFs
Chromosomal Location of Human Ortholog: Xp21.1
Cellular Component: centrosome; Golgi apparatus; photoreceptor outer segment
Molecular Function: guanyl-nucleotide exchange factor activity; protein binding
Biological Process: cilium biogenesis; intracellular protein transport; intraflagellar transport; positive regulation of GTPase activity; response to stimulus; 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
Research Articles on RPGR
1. Although carriers of XLRP are usually asymptomatic or have a mild disease of late onset, the proband presented here exhibited an early-onset, aggressive form of the disease.
Precautions
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