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Protein Page:
RPGR (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
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. Note: This description may include information from UniProtKB.
Protein type: GEFs, Rab; GEFs
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
Reference #:  Q92834 (UniProtKB)
Alt. Names/Synonyms: COD1; CORDX1; CRD; orf15; PCDX; retinitis pigmentosa 15; retinitis pigmentosa 3 GTPase regulator; retinitis pigmentosa GTPase regulator; RP15; RP3; RPGR; X-linked retinitis pigmentosa GTPase regulator; XLRP3
Gene Symbols: RPGR
Molecular weight: 113,387 Da
Basal Isoelectric point: 4.79  Predict pI for various phosphorylation states
Select Structure to View Below

RPGR

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

► Hide Isoforms
 
0 1 - gap
0 11 T335-p GLGLENFtNHFIPTL
0 10 Y393-p SVATFLPySSLTSGN
0 1 T397 FLPySSLTSGNVLQR
0 2 S418-p RRRERERsPDSFSMR
0 6 S518-p NEKSLKLsPVQKQKK
0 1 - gap
0 1 S866-p INAENVEsKKKtVGD
0 1 T870-p NVEsKKKtVGDDEsV
0 1 S876-p KtVGDDEsVPTGYHs
0 1 S883-p sVPTGYHsKTEGAER
0 1 T891-p KTEGAERtNDDSSAE
0 19 Y916-p EERAICEyNENPKGY
0 11 S961-p FLFKRVPsINQKIVK
0 1 - gap
0 1 - gap
  RPGR iso6  
- gap
T335 GLGLENFTNHFIPTL
Y393 SVATFLPYSSLTSGN
T397 FLPYSSLTSGNVLQR
S418 RRRERERSPDSFSMR
S518 NEKSLKLSPVQKQKK
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
K1101-ub SVKYGKHkTYQKkSV
K1106-ub KHkTYQKkSVTNTQG
  mouse

► Hide Isoforms
 
- gap
T335 GLGMENFTNQFFPTL
- gap
S396-p SFSINDLsPRSSLNR
P417 RRRERERPPCSASMV
S518-p NEKLLDFsPIQKQQN
S535-p TFEKVMEsTPCTENE
- gap
- gap
- gap
- gap
T870 PETEGSETIDITDEK
Y898 LQRALREYNENPKGH
- gap
- gap
- gap
  RPGR iso5  
R17-m1 QGVGQHLrLNRVAPA
T373 GLGMENFTNQFFPTL
- gap
S434 SFSINDLSPRSSLNR
P455 RRRERERPPCSASMV
S556 NEKLLDFSPIQKQQA
- gap
- gap
- gap
- gap
- gap
T615 PETEGSETIDITDEK
Y643 LQRALREYNENPKGH
- gap
- gap
- gap
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