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Collaborative role of two distinct cilium-specific cytoskeletal systems in driving Hedgehog-responsive transcription factor trafficking. bioRxiv. 2024 Oct 01.
Ku PI, Sreeja JS, Chadha A, Williams DS, Engelke MF, Subramanian R. PMID: 39386719; PMCID: PMC11463396.
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PubMed Mentions:
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Ehf controls mammary alveolar lineage differentiation and is a putative suppressor of breast tumorigenesis. Dev Cell. 2024 Aug 05; 59(15):1988-2004.e11.
Nightingale R, Reehorst CM, Vukelic N, Papadopoulos N, Liao Y, Guleria S, Bell C, Vaillant F, Paul S, Luk IY, Dhillon AS, Jenkins LJ, Morrow RJ, Jackling FC, Chand AL, Chisanga D, Chen Y, Williams DS, Anderson RL, Ellis S, Meikle PJ, Shi W, Visvader JE, Pal B, Mariadason JM. PMID: 38781975.
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Translation:
HumansAnimalsCells
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Machine learning-based 3D segmentation of mitochondria in polarized epithelial cells. Mitochondrion. 2024 May; 76:101882.
Hultgren NW, Zhou T, Williams DS. PMID: 38599302.
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Translation:
HumansCells
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Expression of two major isoforms of MYO7A in the retina: Considerations for gene therapy of Usher syndrome type 1B. Vision Res. 2023 11; 212:108311.
Gilmore WB, Hultgren NW, Chadha A, Barocio SB, Zhang J, Kutsyr O, Flores-Bellver M, Canto-Soler MV, Williams DS. PMID: 37586294; PMCID: PMC10984346.
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Translation:
HumansAnimals
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Three-Dimensional Ultrastructure of the Normal Rod Photoreceptor Synapse and Degenerative Changes Induced by Retinal Detachment. J Neurosci. 2023 07 26; 43(30):5468-5482.
Torten G, Fisher SK, Linberg KA, Luna G, Perkins G, Ellisman MH, Williams DS. PMID: 37414561; PMCID: PMC10376940.
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PubMed Mentions:
1 Fields:
Translation:
AnimalsCells
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Cones and cone pathways remain functional in advanced retinal degeneration. Curr Biol. 2023 04 24; 33(8):1513-1522.e4.
Ellis EM, Paniagua AE, Scalabrino ML, Thapa M, Rathinavelu J, Jiao Y, Williams DS, Field GD, Fain GL, Sampath AP. PMID: 36977418; PMCID: PMC10133175.
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PubMed Mentions:
8 Fields:
Translation:
HumansCells
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Spatiotemporal Live-Cell Analysis of Photoreceptor Outer Segment Membrane Ingestion by the Retinal Pigment Epithelium Reveals Actin-Regulated Scission. J Neurosci. 2023 04 12; 43(15):2653-2664.
Umapathy A, Torten G, Paniagua AE, Chung J, Tomlinson M, Lim C, Williams DS. PMID: 36878726; PMCID: PMC10089248.
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PubMed Mentions:
4 Fields:
Translation:
AnimalsCells
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Revisiting the Daily Timing of POS Phagocytosis. Adv Exp Med Biol. 2023; 1415:515-519.
Paniagua AE, Sabharwal HS, Kethu K, Chang AW, Williams DS. PMID: 37440080.
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PubMed Mentions:
1 Fields:
Translation:
Cells
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Vitamin B3, nicotinamide, enhances mitochondrial metabolism to promote differentiation of the retinal pigment epithelium. J Biol Chem. 2022 09; 298(9):102286.
Hazim RA, Paniagua AE, Tang L, Yang K, Kim KKO, Stiles L, Divakaruni AS, Williams DS. PMID: 35868562; PMCID: PMC9396405.
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PubMed Mentions:
11 Fields:
Translation:
HumansAnimalsCells
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Cell culture models to study retinal pigment epithelium-related pathogenesis in age-related macular degeneration. Exp Eye Res. 2022 09; 222:109170.
Bharti K, den Hollander AI, Lakkaraju A, Sinha D, Williams DS, Finnemann SC, Bowes-Rickman C, Malek G, D'Amore PA. PMID: 35835183; PMCID: PMC9444976.
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PubMed Mentions:
24 Fields:
Translation:
HumansAnimalsCells
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Microtubule Motor Transport of Organelles in a Specialized Epithelium: The RPE. Front Cell Dev Biol. 2022; 10:852468.
Hazim RA, Williams DS. PMID: 35309899; PMCID: PMC8930850.
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PubMed Mentions:
3
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Deficiency in Lyst function leads to accumulation of secreted proteases and reduced retinal adhesion. PLoS One. 2022; 17(3):e0254469.
Ji X, Zhao L, Umapathy A, Fitzmaurice B, Wang J, Williams DS, Chang B, Naggert JK, Nishina PM. PMID: 35239671; PMCID: PMC8893605.
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PubMed Mentions:
2 Fields:
Translation:
AnimalsCells
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The ApoA-I mimetic peptide 4F attenuates in vitro replication of SARS-CoV-2, associated apoptosis, oxidative stress and inflammation in epithelial cells. Virulence. 2021 12; 12(1):2214-2227.
Kelesidis T, Madhav S, Petcherski A, Cristelle H, O'Connor E, Hultgren NW, Ritou E, Williams DS, Shirihai OS, Reddy ST. PMID: 34494942; PMCID: PMC8437485.
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PubMed Mentions:
7 Fields:
Translation:
HumansAnimalsCells
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Comparison of Ciliary Targeting of Two Rhodopsin-Like GPCRs: Role of C-Terminal Localization Sequences in Relation to Cilium Type. J Neurosci. 2021 09 08; 41(36):7514-7531.
Chadha A, Paniagua AE, Williams DS. PMID: 34301828; PMCID: PMC8425976.
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PubMed Mentions:
4 Fields:
Translation:
HumansAnimalsCells
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Key Role for CRB2 in the Maintenance of Apicobasal Polarity in Retinal Pigment Epithelial Cells. Front Cell Dev Biol. 2021; 9:701853.
Paniagua AE, Segurado A, Dolón JF, Esteve-Rudd J, Velasco A, Williams DS, Lillo C. PMID: 34262913; PMCID: PMC8273544.
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PubMed Mentions:
4
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Host IL11 Signaling Suppresses CD4+ T cell-Mediated Antitumor Responses to Colon Cancer in Mice. Cancer Immunol Res. 2021 07; 9(7):735-747.
Huynh J, Baloyan D, Chisanga D, Shi W, O'Brien M, Afshar-Sterle S, Alorro M, Pang L, Williams DS, Parslow AC, Thilakasiri P, Eissmann MF, Boon L, Masson F, Chand AL, Ernst M. PMID: 33906864.
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PubMed Mentions:
15 Fields:
Translation:
HumansAnimalsCells
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A human model of Batten disease shows role of CLN3 in phagocytosis at the photoreceptor-RPE interface. Commun Biol. 2021 02 05; 4(1):161.
Tang C, Han J, Dalvi S, Manian K, Winschel L, Volland S, Soto CA, Galloway CA, Spencer W, Roll M, Milliner C, Bonilha VL, Johnson TB, Latchney L, Weimer JM, Augustine EF, Mink JW, Gullapalli VK, Chung M, Williams DS, Singh R. PMID: 33547385; PMCID: PMC7864947.
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PubMed Mentions:
15 Translation:
HumansCells
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Slug regulates the Dll4-Notch-VEGFR2 axis to control endothelial cell activation and angiogenesis. Nat Commun. 2020 10 26; 11(1):5400.
Hultgren NW, Fang JS, Ziegler ME, Ramirez RN, Phan DTT, Hatch MMS, Welch-Reardon KM, Paniagua AE, Kim LS, Shon NN, Williams DS, Mortazavi A, Hughes CCW. PMID: 33106502; PMCID: PMC7588439.
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PubMed Mentions:
44 Fields:
Translation:
HumansAnimalsCells
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Microtubule motor transport in the delivery of melanosomes to the actin-rich apical domain of the retinal pigment epithelium. J Cell Sci. 2020 08 04; 133(15).
Jiang M, Paniagua AE, Volland S, Wang H, Balaji A, Li DG, Lopes VS, Burgess BL, Williams DS. PMID: 32661088; PMCID: PMC7420818.
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PubMed Mentions:
12 Fields:
Translation:
Cells
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Quantification of cristae architecture reveals time-dependent characteristics of individual mitochondria. Life Sci Alliance. 2020 07; 3(7).
Segawa M, Wolf DM, Hultgren NW, Williams DS, van der Bliek AM, Shackelford DB, Liesa M, Shirihai OS. PMID: 32499316; PMCID: PMC7283135.
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PubMed Mentions:
21 Fields:
Translation:
HumansCells
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The cell biology of the retinal pigment epithelium. Prog Retin Eye Res. 2020 Feb 24; 100846.
Lakkaraju A, Umapathy A, Tan LX, Daniele L, Philp NJ, Boesze-Battaglia K, Williams DS. PMID: 32105772; PMCID: PMC8941496.
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PubMed Mentions:
146 Fields:
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Multistep peripherin-2/rds self-assembly drives membrane curvature for outer segment disk architecture and photoreceptor viability. Proc Natl Acad Sci U S A. 2020 02 25; 117(8):4400-4410.
Milstein ML, Cavanaugh BL, Roussey NM, Volland S, Williams DS, Goldberg AFX. PMID: 32041874; PMCID: PMC7049111.
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PubMed Mentions:
14 Fields:
Translation:
HumansAnimalsCells
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Peroxisome turnover and diurnal modulation of antioxidant activity in retinal pigment epithelia utilizes microtubule-associated protein 1 light chain 3B (LC3B). Am J Physiol Cell Physiol. 2019 12 01; 317(6):C1194-C1204.
Daniele LL, Caughey J, Volland S, Sharp RC, Dhingra A, Williams DS, Philp NJ, Boesze-Battaglia K. PMID: 31577510; PMCID: PMC6962520.
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PubMed Mentions:
10 Fields:
Translation:
HumansAnimalsCells
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The route of the visual receptor rhodopsin along the cilium. J Cell Sci. 2019 05 15; 132(10).
Chadha A, Volland S, Baliaouri NV, Tran EM, Williams DS. PMID: 30975916; PMCID: PMC6550008.
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PubMed Mentions:
10 Fields:
Translation:
AnimalsCells
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Correction to: Differentiation of RPE cells from integration-free iPS cells and their cell biological characterization. Stem Cell Res Ther. 2019 Feb 12; 10(1):52.
Hazim RA, Karumbayaram S, Jiang M, Dimashkie A, Lopes VS, Li D, Burgess BL, Vijayaraj P, Alva-Ornelas JA, Zack JA, Kohn DB, Gomperts BN, Pyle AD, Lowry WE, Williams DS. PMID: 30755264; PMCID: PMC6373121.
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PubMed Mentions: Fields:
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Live Imaging of Organelle Motility in RPE Flatmounts. Adv Exp Med Biol. 2019; 1185:389-393.
Umapathy A, Williams DS. PMID: 31884643.
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PubMed Mentions:
3 Fields:
Translation:
HumansCells
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Rapid differentiation of the human RPE cell line, ARPE-19, induced by nicotinamide. Exp Eye Res. 2019 02; 179:18-24.
Hazim RA, Volland S, Yen A, Burgess BL, Williams DS. PMID: 30336127; PMCID: PMC6360117.
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PubMed Mentions:
34 Fields:
Translation:
HumansCells
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Defective phagosome motility and degradation in cell nonautonomous RPE pathogenesis of a dominant macular degeneration. Proc Natl Acad Sci U S A. 2018 05 22; 115(21):5468-5473.
Esteve-Rudd J, Hazim RA, Diemer T, Paniagua AE, Volland S, Umapathy A, Williams DS. PMID: 29735674; PMCID: PMC6003516.
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PubMed Mentions:
23 Fields:
Translation:
HumansAnimalsCells
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Preservation of Photoreceptor Nanostructure for Electron Tomography Using Transcardiac Perfusion Followed by High-Pressure Freezing and Freeze-Substitution. Adv Exp Med Biol. 2018; 1074:603-607.
Volland S, Williams DS. PMID: 29721993; PMCID: PMC6501585.
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PubMed Mentions:
4 Fields:
Translation:
AnimalsCells
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Cell Culture Analysis of the Phagocytosis of Photoreceptor Outer Segments by Primary Mouse RPE Cells. Methods Mol Biol. 2018; 1753:63-71.
Hazim RA, Williams DS. PMID: 29564781; PMCID: PMC6531314.
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PubMed Mentions:
4 Fields:
Translation:
AnimalsCells
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Differentiation of RPE cells from integration-free iPS cells and their cell biological characterization. Stem Cell Res Ther. 2017 10 02; 8(1):217.
Hazim RA, Karumbayaram S, Jiang M, Dimashkie A, Lopes VS, Li D, Burgess BL, Vijayaraj P, Alva-Ornelas JA, Zack JA, Kohn DB, Gomperts BN, Pyle AD, Lowry WE, Williams DS. PMID: 28969679; PMCID: PMC5625837.
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PubMed Mentions:
34 Fields:
Translation:
HumansAnimalsCells
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Drusen in patient-derived hiPSC-RPE models of macular dystrophies. Proc Natl Acad Sci U S A. 2017 09 26; 114(39):E8214-E8223.
Galloway CA, Dalvi S, Hung SSC, MacDonald LA, Latchney LR, Wong RCB, Guymer RH, Mackey DA, Williams DS, Chung MM, Gamm DM, Pébay A, Hewitt AW, Singh R. PMID: 28878022; PMCID: PMC5625924.
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PubMed Mentions:
57 Fields:
Translation:
HumansCells
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Molecular basis for photoreceptor outer segment architecture. Prog Retin Eye Res. 2016 11; 55:52-81.
Goldberg AF, Moritz OL, Williams DS. PMID: 27260426; PMCID: PMC5112118.
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PubMed Mentions:
101 Fields:
Translation:
HumansAnimalsCells
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Live-Cell Imaging of Phagosome Motility in Primary Mouse RPE Cells. Adv Exp Med Biol. 2016; 854:751-5.
Hazim R, Jiang M, Esteve-Rudd J, Diemer T, Lopes VS, Williams DS. PMID: 26427485; PMCID: PMC8983022.
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PubMed Mentions:
3 Fields:
Translation:
AnimalsCells
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Three-dimensional organization of nascent rod outer segment disk membranes. Proc Natl Acad Sci U S A. 2015 Dec 01; 112(48):14870-5.
Volland S, Hughes LC, Kong C, Burgess BL, Linberg KA, Luna G, Zhou ZH, Fisher SK, Williams DS. PMID: 26578801; PMCID: PMC4672767.
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PubMed Mentions:
57 Fields:
Translation:
AnimalsCells
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Microtubule motors transport phagosomes in the RPE, and lack of KLC1 leads to AMD-like pathogenesis. J Cell Biol. 2015 Aug 17; 210(4):595-611.
Jiang M, Esteve-Rudd J, Lopes VS, Diemer T, Lillo C, Rump A, Williams DS. PMID: 26261180; PMCID: PMC4539993.
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PubMed Mentions:
52 Fields:
Translation:
AnimalsCells
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A comparison of some organizational characteristics of the mouse central retina and the human macula. PLoS One. 2015; 10(4):e0125631.
Volland S, Esteve-Rudd J, Hoo J, Yee C, Williams DS. PMID: 25923208; PMCID: PMC4414478.
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PubMed Mentions:
111 Fields:
Translation:
HumansAnimals
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Gene Therapy for the Retinal Degeneration of Usher Syndrome Caused by Mutations in MYO7A. Cold Spring Harb Perspect Med. 2015 Jan 20; 5(6).
Lopes VS, Williams DS. PMID: 25605753; PMCID: PMC4448706.
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PubMed Mentions:
8 Fields:
Translation:
HumansAnimalsCells
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Spata7 is a retinal ciliopathy gene critical for correct RPGRIP1 localization and protein trafficking in the retina. Hum Mol Genet. 2015 Mar 15; 24(6):1584-601.
Eblimit A, Nguyen TM, Chen Y, Esteve-Rudd J, Zhong H, Letteboer S, Van Reeuwijk J, Simons DL, Ding Q, Wu KM, Li Y, Van Beersum S, Moayedi Y, Xu H, Pickard P, Wang K, Gan L, Wu SM, Williams DS, Mardon G, Roepman R, Chen R. PMID: 25398945; PMCID: PMC4351378.
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PubMed Mentions:
33 Fields:
Translation:
HumansAnimalsCells
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The Contribution of Melanoregulin to Microtubule-Associated Protein 1 Light Chain 3 (LC3) Associated Phagocytosis in Retinal Pigment Epithelium. Mol Neurobiol. 2015 Dec; 52(3):1135-1151.
Frost LS, Lopes VS, Bragin A, Reyes-Reveles J, Brancato J, Cohen A, Mitchell CH, Williams DS, Boesze-Battaglia K. PMID: 25301234; PMCID: PMC5531606.
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PubMed Mentions:
40 Fields:
Translation:
HumansAnimalsCells
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Mutations in EMP2 cause childhood-onset nephrotic syndrome. Am J Hum Genet. 2014 Jun 05; 94(6):884-90.
Gee HY, Ashraf S, Wan X, Vega-Warner V, Esteve-Rudd J, Lovric S, Fang H, Hurd TW, Sadowski CE, Allen SJ, Otto EA, Korkmaz E, Washburn J, Levy S, Williams DS, Bakkaloglu SA, Zolotnitskaya A, Ozaltin F, Zhou W, Hildebrandt F. PMID: 24814193; PMCID: PMC4121470.
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PubMed Mentions:
61 Fields:
Translation:
HumansAnimalsCells
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Distinct functions for IFT140 and IFT20 in opsin transport. Cytoskeleton (Hoboken). 2014 May; 71(5):302-10.
Crouse JA, Lopes VS, Sanagustin JT, Keady BT, Williams DS, Pazour GJ. PMID: 24619649; PMCID: PMC4173073.
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PubMed Mentions:
32 Fields:
Translation:
AnimalsCells
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UV irradiation accelerates amyloid precursor protein (APP) processing and disrupts APP axonal transport. J Neurosci. 2014 Feb 26; 34(9):3320-39.
Almenar-Queralt A, Falzone TL, Shen Z, Lillo C, Killian RL, Arreola AS, Niederst ED, Ng KS, Kim SN, Briggs SP, Williams DS, Goldstein LS. PMID: 24573290; PMCID: PMC3935090.
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PubMed Mentions:
14 Fields:
Translation:
HumansAnimalsCells
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Assessment of different virus-mediated approaches for retinal gene therapy of Usher 1B. Adv Exp Med Biol. 2014; 801:725-31.
Lopes VS, Diemer T, Williams DS. PMID: 24664764.
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PubMed Mentions:
1 Fields:
Translation:
HumansAnimalsCells
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In vivo and in vitro monitoring of phagosome maturation in retinal pigment epithelium cells. Adv Exp Med Biol. 2014; 801:85-90.
Esteve-Rudd J, Lopes VS, Jiang M, Williams DS. PMID: 24664684.
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PubMed Mentions:
15 Fields:
Translation:
AnimalsCells
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ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption. J Clin Invest. 2013 Dec; 123(12):5179-89.
Ashraf S, Gee HY, Woerner S, Xie LX, Vega-Warner V, Lovric S, Fang H, Song X, Cattran DC, Avila-Casado C, Paterson AD, Nitschké P, Bole-Feysot C, Cochat P, Esteve-Rudd J, Haberberger B, Allen SJ, Zhou W, Airik R, Otto EA, Barua M, Al-Hamed MH, Kari JA, Evans J, Bierzynska A, Saleem MA, Böckenhauer D, Kleta R, El Desoky S, Hacihamdioglu DO, Gok F, Washburn J, Wiggins RC, Choi M, Lifton RP, Levy S, Han Z, Salviati L, Prokisch H, Williams DS, Pollak M, Clarke CF, Pei Y, Antignac C, Hildebrandt F. PMID: 24270420; PMCID: PMC3859425.
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PubMed Mentions:
181 Fields:
Translation:
HumansAnimalsCells
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A three-dimensional culture method to expand limbal stem/progenitor cells. Tissue Eng Part C Methods. 2014 May; 20(5):393-400.
Mei H, González S, Nakatsu MN, Baclagon ER, Lopes VS, Williams DS, Deng SX. PMID: 24047104; PMCID: PMC4005490.
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PubMed Mentions:
11 Fields:
Translation:
HumansAnimalsCells
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ZMYND10 is mutated in primary ciliary dyskinesia and interacts with LRRC6. Am J Hum Genet. 2013 Aug 08; 93(2):336-45.
Zariwala MA, Gee HY, Kurkowiak M, Al-Mutairi DA, Leigh MW, Hurd TW, Hjeij R, Dell SD, Chaki M, Dougherty GW, Adan M, Spear PC, Esteve-Rudd J, Loges NT, Rosenfeld M, Diaz KA, Olbrich H, Wolf WE, Sheridan E, Batten TF, Halbritter J, Porath JD, Kohl S, Lovric S, Hwang DY, Pittman JE, Burns KA, Ferkol TW, Sagel SD, Olivier KN, Morgan LC, Werner C, Raidt J, Pennekamp P, Sun Z, Zhou W, Airik R, Natarajan S, Allen SJ, Amirav I, Wieczorek D, Landwehr K, Nielsen K, Schwerk N, Sertic J, Köhler G, Washburn J, Levy S, Fan S, Koerner-Rettberg C, Amselem S, Williams DS, Mitchell BJ, Drummond IA, Otto EA, Omran H, Knowles MR, Hildebrandt F. PMID: 23891469; PMCID: PMC3738827.
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PubMed Mentions:
97 Fields:
Translation:
HumansAnimalsCells
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Loss of melanoregulin (MREG) enhances cathepsin-D secretion by the retinal pigment epithelium. Vis Neurosci. 2013 May; 30(3):55-64.
Frost LS, Lopes VS, Stefano FP, Bragin A, Williams DS, Mitchell CH, Boesze-Battaglia K. PMID: 23611523; PMCID: PMC3912860.
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PubMed Mentions:
6 Fields:
Translation:
AnimalsCells
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Live-cell imaging evidence for the ciliary transport of rod photoreceptor opsin by heterotrimeric kinesin-2. J Neurosci. 2012 Aug 01; 32(31):10587-93.
Trivedi D, Colin E, Louie CM, Williams DS. PMID: 22855808; PMCID: PMC3428073.
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PubMed Mentions:
39 Fields:
Translation:
HumansAnimalsCells
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Elevated PGC-1α activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS. Cell Metab. 2012 May 02; 15(5):778-86.
Da Cruz S, Parone PA, Lopes VS, Lillo C, McAlonis-Downes M, Lee SK, Vetto AP, Petrosyan S, Marsala M, Murphy AN, Williams DS, Spiegelman BM, Cleveland DW. PMID: 22560226; PMCID: PMC3565468.
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PubMed Mentions:
89 Fields:
Translation:
HumansAnimalsCells
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Functional and morphological analysis of the subretinal injection of retinal pigment epithelium cells. Vis Neurosci. 2012 Mar; 29(2):83-93.
Engelhardt M, Tosha C, Lopes VS, Chen B, Nguyen L, Nusinowitz S, Williams DS. PMID: 22391151; PMCID: PMC3694443.
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PubMed Mentions:
13 Fields:
Translation:
AnimalsCells
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Gene therapy strategies for Usher syndrome type 1B. Adv Exp Med Biol. 2012; 723:235-42.
Williams DS, Lopes VS. PMID: 22183338.
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PubMed Mentions:
1 Fields:
Translation:
HumansAnimalsCells
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Essential role of ELOVL4 protein in very long chain fatty acid synthesis and retinal function. J Biol Chem. 2012 Mar 30; 287(14):11469-80.
Harkewicz R, Du H, Tong Z, Alkuraya H, Bedell M, Sun W, Wang X, Hsu YH, Esteve-Rudd J, Hughes G, Su Z, Zhang M, Lopes VS, Molday RS, Williams DS, Dennis EA, Zhang K. PMID: 22199362; PMCID: PMC3322886.
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PubMed Mentions:
48 Fields:
Translation:
AnimalsCells
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Retinal disease course in Usher syndrome 1B due to MYO7A mutations. Invest Ophthalmol Vis Sci. 2011 Oct 07; 52(11):7924-36.
Jacobson SG, Cideciyan AV, Gibbs D, Sumaroka A, Roman AJ, Aleman TS, Schwartz SB, Olivares MB, Russell RC, Steinberg JD, Kenna MA, Kimberling WJ, Rehm HL, Williams DS. PMID: 21873662; PMCID: PMC3263772.
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PubMed Mentions:
33 Fields:
Translation:
HumansCells
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The many different cellular functions of MYO7A in the retina. Biochem Soc Trans. 2011 Oct; 39(5):1207-10.
Williams DS, Lopes VS. PMID: 21936790; PMCID: PMC3703834.
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PubMed Mentions:
32 Fields:
Translation:
HumansAnimalsCells
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Intestinal epithelial-specific PTEN inactivation results in tumor formation. Am J Physiol Gastrointest Liver Physiol. 2011 Nov; 301(5):G856-64.
Byun DS, Ahmed N, Nasser S, Shin J, Al-Obaidi S, Goel S, Corner GA, Wilson AJ, Flanagan DJ, Williams DS, Augenlicht LH, Vincan E, Mariadason JM. PMID: 21836055; PMCID: PMC3220321.
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PubMed Mentions:
13 Fields:
Translation:
AnimalsCells
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Harmonin (Ush1c) is required in zebrafish Müller glial cells for photoreceptor synaptic development and function. Dis Model Mech. 2011 Nov; 4(6):786-800.
Phillips JB, Blanco-Sanchez B, Lentz JJ, Tallafuss A, Khanobdee K, Sampath S, Jacobs ZG, Han PF, Mishra M, Titus TA, Williams DS, Keats BJ, Washbourne P, Westerfield M. PMID: 21757509; PMCID: PMC3209648.
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PubMed Mentions:
38 Fields:
Translation:
AnimalsCells
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The Usher 1B protein, MYO7A, is required for normal localization and function of the visual retinoid cycle enzyme, RPE65. Hum Mol Genet. 2011 Jul 01; 20(13):2560-70.
Lopes VS, Gibbs D, Libby RT, Aleman TS, Welch DL, Lillo C, Jacobson SG, Radu RA, Steel KP, Williams DS. PMID: 21493626; PMCID: PMC3110002.
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PubMed Mentions:
40 Fields:
Translation:
HumansAnimalsCells
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Dysfunction of heterotrimeric kinesin-2 in rod photoreceptor cells and the role of opsin mislocalization in rapid cell death. Mol Biol Cell. 2010 Dec; 21(23):4076-88.
Lopes VS, Jimeno D, Khanobdee K, Song X, Chen B, Nusinowitz S, Williams DS. PMID: 20926680; PMCID: PMC2993738.
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PubMed Mentions:
34 Fields:
Translation:
AnimalsCells
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Candidate exome capture identifies mutation of SDCCAG8 as the cause of a retinal-renal ciliopathy. Nat Genet. 2010 Oct; 42(10):840-50.
Otto EA, Hurd TW, Airik R, Chaki M, Zhou W, Stoetzel C, Patil SB, Levy S, Ghosh AK, Murga-Zamalloa CA, van Reeuwijk J, Letteboer SJ, Sang L, Giles RH, Liu Q, Coene KL, Estrada-Cuzcano A, Collin RW, McLaughlin HM, Held S, Kasanuki JM, Ramaswami G, Conte J, Lopez I, Washburn J, Macdonald J, Hu J, Yamashita Y, Maher ER, Guay-Woodford LM, Neumann HP, Obermüller N, Koenekoop RK, Bergmann C, Bei X, Lewis RA, Katsanis N, Lopes V, Williams DS, Lyons RH, Dang CV, Brito DA, Dias MB, Zhang X, Cavalcoli JD, Nürnberg G, Nürnberg P, Pierce EA, Jackson PK, Antignac C, Saunier S, Roepman R, Dollfus H, Khanna H, Hildebrandt F. PMID: 20835237; PMCID: PMC2947620.
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PubMed Mentions:
195 Fields:
Translation:
HumansAnimalsCells
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Molecular signature of primary retinal pigment epithelium and stem-cell-derived RPE cells. Hum Mol Genet. 2010 Nov 01; 19(21):4229-38.
Liao JL, Yu J, Huang K, Hu J, Diemer T, Ma Z, Dvash T, Yang XJ, Travis GH, Williams DS, Bok D, Fan G. PMID: 20709808; PMCID: PMC3115666.
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94 Fields:
Translation:
HumansCells
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AHI1 is required for photoreceptor outer segment development and is a modifier for retinal degeneration in nephronophthisis. Nat Genet. 2010 Feb; 42(2):175-80.
Louie CM, Caridi G, Lopes VS, Brancati F, Kispert A, Lancaster MA, Schlossman AM, Otto EA, Leitges M, Gröne HJ, Lopez I, Gudiseva HV, O'Toole JF, Vallespin E, Ayyagari R, Ayuso C, Cremers FP, den Hollander AI, Koenekoop RK, Dallapiccola B, Ghiggeri GM, Hildebrandt F, Valente EM, Williams DS, Gleeson JG. PMID: 20081859; PMCID: PMC2884967.
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PubMed Mentions:
120 Fields:
Translation:
HumansAnimalsCells
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Ciliary transport of opsin. Adv Exp Med Biol. 2010; 664:185-91.
Trivedi D, Williams DS. PMID: 20238016; PMCID: PMC2933454.
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PubMed Mentions:
5 Fields:
Translation:
HumansAnimalsCells
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A novel allele of myosin VIIa reveals a critical function for the C-terminal FERM domain for melanosome transport in retinal pigment epithelial cells. J Neurosci. 2009 Dec 16; 29(50):15810-8.
Schwander M, Lopes V, Sczaniecka A, Gibbs D, Lillo C, Delano D, Tarantino LM, Wiltshire T, Williams DS, Müller U. PMID: 20016096; PMCID: PMC2834289.
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PubMed Mentions:
24 Fields:
Translation:
AnimalsCells
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Trafficking of membrane proteins to cone but not rod outer segments is dependent on heterotrimeric kinesin-II. J Neurosci. 2009 Nov 11; 29(45):14287-98.
Avasthi P, Watt CB, Williams DS, Le YZ, Li S, Chen CK, Marc RE, Frederick JM, Baehr W. PMID: 19906976; PMCID: PMC2788486.
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PubMed Mentions:
55 Fields:
Translation:
AnimalsCells
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Oxaloacetate supplementation increases lifespan in Caenorhabditis elegans through an AMPK/FOXO-dependent pathway. Aging Cell. 2009 Dec; 8(6):765-8.
Williams DS, Cash A, Hamadani L, Diemer T. PMID: 19793063; PMCID: PMC2988682.
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PubMed Mentions:
52 Fields:
Translation:
AnimalsCells
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Sunday driver interacts with two distinct classes of axonal organelles. J Biol Chem. 2009 Dec 11; 284(50):34628-39.
Abe N, Almenar-Queralt A, Lillo C, Shen Z, Lozach J, Briggs SP, Williams DS, Goldstein LS, Cavalli V. PMID: 19801628; PMCID: PMC2787325.
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PubMed Mentions:
40 Fields:
Translation:
AnimalsCells
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Function of MYO7A in the human RPE and the validity of shaker1 mice as a model for Usher syndrome 1B. Invest Ophthalmol Vis Sci. 2010 Feb; 51(2):1130-5.
Gibbs D, Diemer T, Khanobdee K, Hu J, Bok D, Williams DS. PMID: 19643958; PMCID: PMC2868451.
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PubMed Mentions:
25 Fields:
Translation:
HumansAnimalsCells
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Axonal stress kinase activation and tau misbehavior induced by kinesin-1 transport defects. J Neurosci. 2009 May 06; 29(18):5758-67.
Falzone TL, Stokin GB, Lillo C, Rodrigues EM, Westerman EL, Williams DS, Goldstein LS. PMID: 19420244; PMCID: PMC3849468.
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PubMed Mentions:
57 Fields:
Translation:
AnimalsCells
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Harmonin in the murine retina and the retinal phenotypes of Ush1c-mutant mice and human USH1C. Invest Ophthalmol Vis Sci. 2009 Aug; 50(8):3881-9.
Williams DS, Aleman TS, Lillo C, Lopes VS, Hughes LC, Stone EM, Jacobson SG. PMID: 19324851; PMCID: PMC2893298.
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PubMed Mentions:
23 Fields:
Translation:
HumansAnimalsCells
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Retinal pigment epithelium defects in humans and mice with mutations in MYO7A: imaging melanosome-specific autofluorescence. Invest Ophthalmol Vis Sci. 2009 Sep; 50(9):4386-93.
Gibbs D, Cideciyan AV, Jacobson SG, Williams DS. PMID: 19324852; PMCID: PMC2884175.
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37 Fields:
Translation:
HumansAnimalsCells
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Melanoregulin (MREG) modulates lysosome function in pigment epithelial cells. J Biol Chem. 2009 Apr 17; 284(16):10877-89.
Damek-Poprawa M, Diemer T, Lopes VS, Lillo C, Harper DC, Marks MS, Wu Y, Sparrow JR, Rachel RA, Williams DS, Boesze-Battaglia K. PMID: 19240024; PMCID: PMC2667774.
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PubMed Mentions:
33 Fields:
Translation:
HumansAnimalsCells
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Amyloid precursor protein-induced axonopathies are independent of amyloid-beta peptides. Hum Mol Genet. 2008 Nov 15; 17(22):3474-86.
Stokin GB, Almenar-Queralt A, Gunawardena S, Rodrigues EM, Falzone T, Kim J, Lillo C, Mount SL, Roberts EA, McGowan E, Williams DS, Goldstein LS. PMID: 18694898; PMCID: PMC2722897.
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PubMed Mentions:
44 Fields:
Translation:
HumansAnimalsCells
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Mutant prominin 1 found in patients with macular degeneration disrupts photoreceptor disk morphogenesis in mice. J Clin Invest. 2008 Aug; 118(8):2908-16.
Yang Z, Chen Y, Lillo C, Chien J, Yu Z, Michaelides M, Klein M, Howes KA, Li Y, Kaminoh Y, Chen H, Zhao C, Chen Y, Al-Sheikh YT, Karan G, Corbeil D, Escher P, Kamaya S, Li C, Johnson S, Frederick JM, Zhao Y, Wang C, Cameron DJ, Huttner WB, Schorderet DF, Munier FL, Moore AT, Birch DG, Baehr W, Hunt DM, Williams DS, Zhang K. PMID: 18654668; PMCID: PMC2483685.
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PubMed Mentions:
140 Fields:
Translation:
HumansAnimalsCells
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Usher syndromes due to MYO7A, PCDH15, USH2A or GPR98 mutations share retinal disease mechanism. Hum Mol Genet. 2008 Aug 01; 17(15):2405-15.
Jacobson SG, Cideciyan AV, Aleman TS, Sumaroka A, Roman AJ, Gardner LM, Prosser HM, Mishra M, Bech-Hansen NT, Herrera W, Schwartz SB, Liu XZ, Kimberling WJ, Steel KP, Williams DS. PMID: 18463160; PMCID: PMC2733815.
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PubMed Mentions:
59 Fields:
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HumansAnimalsCells
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Serotype-dependent packaging of large genes in adeno-associated viral vectors results in effective gene delivery in mice. J Clin Invest. 2008 May; 118(5):1955-64.
Allocca M, Doria M, Petrillo M, Colella P, Garcia-Hoyos M, Gibbs D, Kim SR, Maguire A, Rex TS, Di Vicino U, Cutillo L, Sparrow JR, Williams DS, Bennett J, Auricchio A. PMID: 18414684; PMCID: PMC2298836.
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PubMed Mentions:
132 Fields:
Translation:
HumansAnimalsCells
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Nrl-knockout mice deficient in Rpe65 fail to synthesize 11-cis retinal and cone outer segments. Invest Ophthalmol Vis Sci. 2008 Mar; 49(3):1126-35.
Feathers KL, Lyubarsky AL, Khan NW, Teofilo K, Swaroop A, Williams DS, Pugh EN, Thompson DA. PMID: 18326740; PMCID: PMC4505835.
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PubMed Mentions:
27 Fields:
Translation:
AnimalsCells
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Analysis of the rate of disk membrane digestion by cultured RPE cells. Adv Exp Med Biol. 2008; 613:321-6.
Diemer T, Gibbs D, Williams DS. PMID: 18188960.
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9 Fields:
Translation:
HumansAnimalsCells
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Usher syndrome: animal models, retinal function of Usher proteins, and prospects for gene therapy. Vision Res. 2008 Feb; 48(3):433-41.
Williams DS. PMID: 17936325; PMCID: PMC2680226.
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PubMed Mentions:
92 Fields:
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HumansAnimals
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Analysis of the linkage of MYRIP and MYO7A to melanosomes by RAB27A in retinal pigment epithelial cells. Cell Motil Cytoskeleton. 2007 Jun; 64(6):474-87.
Klomp AE, Teofilo K, Legacki E, Williams DS. PMID: 17352418.
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20 Fields:
Translation:
AnimalsCells
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The tetraspanin protein peripherin-2 forms a complex with melanoregulin, a putative membrane fusion regulator. Biochemistry. 2007 Feb 06; 46(5):1256-72.
Boesze-Battaglia K, Song H, Sokolov M, Lillo C, Pankoski-Walker L, Gretzula C, Gallagher B, Rachel RA, Jenkins NA, Copeland NG, Morris F, Jacob J, Yeagle P, Williams DS, Damek-Poprawa M. PMID: 17260955; PMCID: PMC4472003.
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PubMed Mentions:
21 Fields:
Translation:
HumansAnimalsCells
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Analysis of kinesin-2 function in photoreceptor cells using synchronous Cre-loxP knockout of Kif3a with RHO-Cre. Invest Ophthalmol Vis Sci. 2006 Nov; 47(11):5039-46.
Jimeno D, Feiner L, Lillo C, Teofilo K, Goldstein LS, Pierce EA, Williams DS. PMID: 17065525; PMCID: PMC1904505.
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PubMed Mentions:
48 Fields:
Translation:
AnimalsCells
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The centrosomal protein nephrocystin-6 is mutated in Joubert syndrome and activates transcription factor ATF4. Nat Genet. 2006 Jun; 38(6):674-81.
Sayer JA, Otto EA, O'Toole JF, Nurnberg G, Kennedy MA, Becker C, Hennies HC, Helou J, Attanasio M, Fausett BV, Utsch B, Khanna H, Liu Y, Drummond I, Kawakami I, Kusakabe T, Tsuda M, Ma L, Lee H, Larson RG, Allen SJ, Wilkinson CJ, Nigg EA, Shou C, Lillo C, Williams DS, Hoppe B, Kemper MJ, Neuhaus T, Parisi MA, Glass IA, Petry M, Kispert A, Gloy J, Ganner A, Walz G, Zhu X, Goldman D, Nurnberg P, Swaroop A, Leroux MR, Hildebrandt F. PMID: 16682973.
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PubMed Mentions:
291 Fields:
Translation:
HumansAnimals
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In-frame deletion in a novel centrosomal/ciliary protein CEP290/NPHP6 perturbs its interaction with RPGR and results in early-onset retinal degeneration in the rd16 mouse. Hum Mol Genet. 2006 Jun 01; 15(11):1847-57.
Chang B, Khanna H, Hawes N, Jimeno D, He S, Lillo C, Parapuram SK, Cheng H, Scott A, Hurd RE, Sayer JA, Otto EA, Attanasio M, O'Toole JF, Jin G, Shou C, Hildebrandt F, Williams DS, Heckenlively JR, Swaroop A. PMID: 16632484; PMCID: PMC1592550.
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PubMed Mentions:
221 Fields:
Translation:
HumansAnimalsCells
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Proteomic analysis of mature melanosomes from the retinal pigmented epithelium. J Proteome Res. 2006 Mar; 5(3):521-9.
Azarian SM, McLeod I, Lillo C, Gibbs D, Yates JR, Williams DS. PMID: 16512666.
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PubMed Mentions:
13 Fields:
Translation:
AnimalsCells
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Roles and interactions of usher 1 proteins in the outer retina. Adv Exp Med Biol. 2006; 572:341-8.
Lillo C, Kitamoto J, Williams DS. PMID: 17249594.
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PubMed Mentions:
7 Fields:
Translation:
HumansAnimalsCells
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Kinesin-2 and photoreceptor cell death: requirement of motor subunits. Exp Eye Res. 2006 Feb; 82(2):351-3.
Jimeno D, Lillo C, Roberts EA, Goldstein LS, Williams DS. PMID: 16337628.
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PubMed Mentions:
13 Fields:
Translation:
AnimalsCells
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Carboxypeptidase E is required for normal synaptic transmission from photoreceptors to the inner retina. J Neurochem. 2005 Dec; 95(5):1351-62.
Zhu X, Wu K, Rife L, Cawley NX, Brown B, Adams T, Teofilo K, Lillo C, Williams DS, Loh YP, Craft CM. PMID: 16219026.
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PubMed Mentions:
23 Fields:
Translation:
AnimalsCells
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RPGR-ORF15, which is mutated in retinitis pigmentosa, associates with SMC1, SMC3, and microtubule transport proteins. J Biol Chem. 2005 Sep 30; 280(39):33580-7.
Khanna H, Hurd TW, Lillo C, Shu X, Parapuram SK, He S, Akimoto M, Wright AF, Margolis B, Williams DS, Swaroop A. PMID: 16043481; PMCID: PMC1249479.
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PubMed Mentions:
79 Fields:
Translation:
HumansAnimalsCells
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Myosin VI is required for normal retinal function. Exp Eye Res. 2005 Jul; 81(1):116-20.
Kitamoto J, Libby RT, Gibbs D, Steel KP, Williams DS. PMID: 15978262.
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PubMed Mentions:
8 Fields:
Translation:
AnimalsCells
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Cone-like morphological, molecular, and electrophysiological features of the photoreceptors of the Nrl knockout mouse. Invest Ophthalmol Vis Sci. 2005 Jun; 46(6):2156-67.
Daniele LL, Lillo C, Lyubarsky AL, Nikonov SS, Philp N, Mears AJ, Swaroop A, Williams DS, Pugh EN. PMID: 15914637; PMCID: PMC4505833.
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PubMed Mentions:
141 Fields:
Translation:
AnimalsCells
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Axonopathy and transport deficits early in the pathogenesis of Alzheimer's disease. Science. 2005 Feb 25; 307(5713):1282-8.
Stokin GB, Lillo C, Falzone TL, Brusch RG, Rockenstein E, Mount SL, Raman R, Davies P, Masliah E, Williams DS, Goldstein LS. PMID: 15731448.
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PubMed Mentions:
508 Fields:
Translation:
HumansAnimalsCells
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Nephrocystin-5, a ciliary IQ domain protein, is mutated in Senior-Loken syndrome and interacts with RPGR and calmodulin. Nat Genet. 2005 Mar; 37(3):282-8.
Otto EA, Loeys B, Khanna H, Hellemans J, Sudbrak R, Fan S, Muerb U, O'Toole JF, Helou J, Attanasio M, Utsch B, Sayer JA, Lillo C, Jimeno D, Coucke P, De Paepe A, Reinhardt R, Klages S, Tsuda M, Kawakami I, Kusakabe T, Omran H, Imm A, Tippens M, Raymond PA, Hill J, Beales P, He S, Kispert A, Margolis B, Williams DS, Swaroop A, Hildebrandt F. PMID: 15723066.
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PubMed Mentions:
195 Fields:
Translation:
HumansCells
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Role of myosin VIIa and Rab27a in the motility and localization of RPE melanosomes. J Cell Sci. 2004 Dec 15; 117(Pt 26):6473-83.
Gibbs D, Azarian SM, Lillo C, Kitamoto J, Klomp AE, Steel KP, Libby RT, Williams DS. PMID: 15572405; PMCID: PMC2942070.
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PubMed Mentions:
73 Fields:
Translation:
AnimalsCells
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Myosin Va is required for normal photoreceptor synaptic activity. J Cell Sci. 2004 Sep 01; 117(Pt 19):4509-15.
Libby RT, Lillo C, Kitamoto J, Williams DS, Steel KP. PMID: 15316067.
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PubMed Mentions:
21 Fields:
Translation:
AnimalsCells
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Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron. 2004 Jul 08; 43(1):5-17.
Liu J, Lillo C, Jonsson PA, Vande Velde C, Ward CM, Miller TM, Subramaniam JR, Rothstein JD, Marklund S, Andersen PM, Brännström T, Gredal O, Wong PC, Williams DS, Cleveland DW. PMID: 15233913.
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PubMed Mentions:
210 Fields:
Translation:
HumansAnimalsCells
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Cadherin 23 is a component of the tip link in hair-cell stereocilia. Nature. 2004 Apr 29; 428(6986):950-5.
Siemens J, Lillo C, Dumont RA, Reynolds A, Williams DS, Gillespie PG, Müller U. PMID: 15057245.
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PubMed Mentions:
225 Fields:
Translation:
HumansAnimalsCells
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Cdh23 mutations in the mouse are associated with retinal dysfunction but not retinal degeneration. Exp Eye Res. 2003 Dec; 77(6):731-9.
Libby RT, Kitamoto J, Holme RH, Williams DS, Steel KP. PMID: 14609561.
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PubMed Mentions:
18 Fields:
Translation:
Animals
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Differential spatial and temporal phosphorylation of the visual receptor, rhodopsin, at two primary phosphorylation sites in mice exposed to light. Biochem J. 2003 Sep 01; 374(Pt 2):537-43.
Adams RA, Liu X, Williams DS, Newton AC. PMID: 12809555; PMCID: PMC1223618.
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PubMed Mentions:
7 Fields:
Translation:
HumansAnimalsCells
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Abnormal phagocytosis by retinal pigmented epithelium that lacks myosin VIIa, the Usher syndrome 1B protein. Proc Natl Acad Sci U S A. 2003 May 27; 100(11):6481-6.
Gibbs D, Kitamoto J, Williams DS. PMID: 12743369; PMCID: PMC164472.
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PubMed Mentions:
136 Fields:
Translation:
AnimalsCells
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Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A. J Cell Biol. 2003 Apr 14; 161(1):55-66.
Xia CH, Roberts EA, Her LS, Liu X, Williams DS, Cleveland DW, Goldstein LS. PMID: 12682084; PMCID: PMC2172877.
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PubMed Mentions:
141 Fields:
Translation:
AnimalsCells
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Isolation and culture of primary mouse retinal pigmented epithelial cells. Adv Exp Med Biol. 2003; 533:347-52.
Gibbs D, Williams DS. PMID: 15180284.
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PubMed Mentions:
26 Fields:
Translation:
AnimalsCells
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Screen for usher syndrome 1B mutations in the ovine myosin VIIa gene. Adv Exp Med Biol. 2003; 533:151-5.
Slatter T, Azarian SM, Tebbutt S, Maw M, Williams DS. PMID: 15180259.
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PubMed Mentions: Fields:
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AnimalsCells
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Mouse models for Usher syndrome 1B. Adv Exp Med Biol. 2003; 533:143-50.
Lillo C, Kitamoto J, Liu X, Quint E, Steel KP, Williams DS. PMID: 15180258.
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PubMed Mentions:
16 Fields:
Translation:
Animals
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Transport to the photoreceptor outer segment by myosin VIIa and kinesin II. Vision Res. 2002 Feb; 42(4):455-62.
Williams DS. PMID: 11853761.
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PubMed Mentions:
36 Fields:
Translation:
AnimalsCells
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Actin-based motor properties of native myosin VIIa. J Cell Sci. 2002 Jan 15; 115(Pt 2):445-50.
Udovichenko IP, Gibbs D, Williams DS. PMID: 11839794.
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PubMed Mentions:
37 Fields:
Translation:
AnimalsCells