Anti-RPGR Antibody (CAB1844)

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SKU:
CAB1844
Product type:
Antibody
Reactivity:
Human
Mouse
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology
Frequently bought together:

Description

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Antibody Name:Anti-RPGR Antibody
Antibody SKU:CAB1844
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-260 of human RPGR (NP_001030025.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:Y79, Mouse heart
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-260 of human RPGR (NP_001030025.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:MREP EELM PDSG AVFT FGKS KFAE NNPG KFWF KNDV PVHL SCGD EHSA VVTG NNKL YMFG SNNW GQLG LGSK SAIS KPTC VKAL KPEK VKLA ACGR NHTL VSTE GGNV YATG GNNE GQLG LGDT EERN TFHV ISFF TSEH KIKQ LSAG SNTS AALT EDGR LFMW GDNS EGQI GLKN VSNV CVPQ QVTI GKPV SWIS CGYY HSAF VTTD GELY VFGE PENG KLGL PNQL LGNH RTPQ LVSE IPEK VIQV ACGG EHTV VLTE
Gene ID:6103
Uniprot:Q92834
Cellular Location:Cytoplasm, Golgi apparatus, centrosome, cilium axoneme, cilium basal body, cytoskeleton, flagellum axoneme, microtubule organizing center
Calculated MW:52kDa/70kDa/83kDa/90kDa/113kDa/127kDa
Observed MW:85kDa
Synonyms:RPGR, COD1, CORDX1, CRD, PCDX, RP15, RP3, XLRP3, orf15
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.
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:GEFs; GEFs, Rab

Chromosomal Location of Human Ortholog: Xp21.1

Cellular Component: Golgi apparatus; centrosome; photoreceptor outer segment

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

Biological Process: intracellular protein transport; eye photoreceptor cell development; visual perception; response to stimulus; intraflagellar transport; cilium biogenesis; positive regulation of GTPase activity

Disease: Retinitis Pigmentosa, X-linked, And Sinorespiratory Infections, With Or Without Deafness; Cone-rod Dystrophy, X-linked, 1; Macular Degeneration, X-linked Atrophic; Retinitis Pigmentosa 3

NCBI Summary: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 Code:Q92834
NCBI GenInfo Identifier:23503098
NCBI Gene ID:6103
NCBI Accession:Q92834.2
UniProt Secondary Accession:Q92834,O00702, O00737, Q3KN84, Q8N5T6, Q93039, Q9HD29 Q9UMR1, B1ARN3, E9PE28,
UniProt Related Accession:Q92834
Molecular Weight:1020
NCBI Full Name:X-linked retinitis pigmentosa GTPase regulator
NCBI Synonym Full Names:retinitis pigmentosa GTPase regulator
NCBI Official Symbol:RPGR  
NCBI Official Synonym Symbols:CRD; RP3; COD1; PCDX; RP15; XLRP3; orf15; CORDX1  
NCBI Protein Information:X-linked retinitis pigmentosa GTPase regulator; retinitis pigmentosa 15; retinitis pigmentosa 3 GTPase regulator
UniProt Protein Name:X-linked retinitis pigmentosa GTPase regulator
Protein Family:X-linked retinitis pigmentosa GTPase regulator
UniProt Gene Name:RPGR  
UniProt Entry Name:RPGR_HUMAN
Anti-RPGR Antibody (CAB1844)Western blot analysis of extracts of various cell lines, using RPGR antibody (CAB1844) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST.

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