Product Name
RPGR, cDNA Clone
Full Product Name
RPGR cDNA Clone
Product Gene Name
RPGR cdna clone
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence
atgagggagc cggaagagct gatgcccgat tcgggtgctg tgtttacatt tgggaaaagt aaatttgctg aaaataatcc cggtaaattc tggtttaaaa atgatgtccc tgtacatctt tcatgtggag atgaacattc tgctgttgtt accggaaata ataaacttta catgtttggc agtaacaact ggggtcagtt aggattagga tcaaagtcag ccatcagcaa gccaacatgt gtcaaagctc taaaacctga aaaagtgaaa ttagctgcct gtggaaggaa ccacaccctg gtgtcaacag aaggaggcaa tgtatatgca actggtggaa ataatgaagg acagttgggg cttggtgaca ccgaagaaag aaacactttt catgtaatta gcttttttac atccgagcat aagattaagc agctgtctgc tggatctaat acttcagctg ccctaactga ggatggaaga ctttttatgt ggggtgacaa ttccgaaggg caaattggtt taaaaaatgt aagtaatgtc tgtgtccctc agcaagtgac cattgggaaa cctgtctcct ggatctcttg tggatattac cattcagctt ttgtaacaac agatggtgag ctatatgtgt ttggagaacc tgagaatggg aagttaggtc ttcccaatca gctcctgggc aatcacagaa caccccagct ggtgtctgaa attccggaga aggtgatcca agtagcctgt ggtggagagc atactgtggt tctcacggag aatgctgtgt atacctttgg gctgggacaa tttggtcagc tgggtcttgg cacttttctt tttgaaactt cagaacccaa agtcattgag aatattaggg atcaaacaat aagttatatt tcttgtggag aaaatcacac agctttgata acagatatcg gccttatgta tacttttgga gatggtcgcc acggaaaatt aggacttgga ctggagaatt ttaccaatca cttcattcct actttgtgct ctaatttttt gaggtttata gttaaattgg ttgcttgtgg tggatgtcac atggtagttt ttgctgctcc tcatcgtggt gtggcaaaag aaattgaatt tgatgaaata aatgatactt gcttatctgt ggcgactttt ctgccgtata gcagtttaac ctcaggaaat gtactgcaga ggactctatc agcacgtatg cggcgaagag agagggagag gtctccagat tctttttcaa tgaggagaac actacctcca atagaaggga ctcttggcct ttctgcttgt tttctcccca attcagtctt tccacgatgt tctgagagaa acctccaaga gagtgtctta tctgaacagg acctcatgca gccagaggaa ccagacacac atcatgagcc tgaattccaa tga
Clone Sequence Report
Provided with product shipment
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of RPGR cdna clone 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.
NCBI/Uniprot data below describe general gene information for RPGR. It may not necessarily be applicable to this product.
NCBI Accession #
BC031624
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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
Homo sapiens retinitis pigmentosa GTPase regulator, mRNA
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: protein binding
Biological Process: cilium biogenesis; intracellular protein transport; intraflagellar transport; 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. Profound visual loss occurred by the second decade of life with progression to near no light perception by age 60 in this kindred of X-linked RP associated with the RPGR genotype.
Precautions
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Disclaimer
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