David S Williams

Title(s)Professor-in-Residence, Ophthalmology
SchoolMedicine
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    Other Positions
    Title(s)Professor-in-Residence, Neurobiology


    Collapse Research 
    Collapse Research Activities and Funding
    Exploring the relationship of water flow across the RPE and mutant-MYO7A/Usher 1B
    NIH R21EY031109Jan 1, 2020 - Dec 31, 2021
    Role: Principal Investigator
    FASEB SRC on The Biology and Chemistry of Vision
    NIH R13EY030354Jun 1, 2019 - May 31, 2020
    Role: Principal Investigator
    RPE cell biology, aging, and disease
    NIH R01EY027442Sep 1, 2017 - May 31, 2022
    Role: Principal Investigator
    Photoreceptor disk membrane morphogenesis
    NIH R01EY024667Apr 1, 2015 - Mar 31, 2020
    Role: Co-Principal Investigator
    The Photoreceptor Cilium
    NIH R01EY013408Sep 30, 2001 - Apr 30, 2020
    Role: Principal Investigator
    TRANSMISSION ELECTRON MICROSCOPE
    NIH S10RR008337May 16, 1994 - May 15, 1995
    Role: Principal Investigator
    RPE Cell Biology of Myosin VIIa
    NIH R01EY007042Sep 30, 1986 - Jun 30, 2015
    Role: Principal Investigator
    IN VITRO STUDIES OF CELLULAR PROCESSES IN RODS AND CONES
    NIH R01EY005410Sep 30, 1985 - Sep 29, 1986
    Role: Principal Investigator
    Vision Research Core at UCLA
    NIH P30EY000331Nov 1, 1978 - Jun 30, 2025
    Role: Principal Investigator

    Collapse Bibliographic 
    Collapse Publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help. to make corrections and additions.
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    Altmetrics Details PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
    1. 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.
      View in: PubMed   Mentions:
    2. 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.
      View in: PubMed   Mentions:    Fields:    Translation:HumansAnimalsCells
    3. 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.
      View in: PubMed   Mentions:
    4. 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.
      View in: PubMed   Mentions:
    5. EHF is essential for epidermal and colonic epithelial homeostasis, and suppresses Apc-initiated colonic tumorigenesis. Development. 2021 06 15; 148(12). Reehorst CM, Nightingale R, Luk IY, Jenkins L, Koentgen F, Williams DS, Darido C, Tan F, Anderton H, Chopin M, Schoffer K, Eissmann MF, Buchert M, Mouradov D, Sieber OM, Ernst M, Dhillon AS, Mariadason JM. PMID: 34180969.
      View in: PubMed   Mentions:
    6. 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.
      View in: PubMed   Mentions:
    7. 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.
      View in: PubMed   Mentions: 4     Fields:    Translation:HumansAnimalsCells
    8. 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.
      View in: PubMed   Mentions: 4     Fields:    Translation:Cells
    9. 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.
      View in: PubMed   Mentions: 3     Fields:    Translation:HumansCells
    10. 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.
      View in: PubMed   Mentions: 29     Fields:    
    11. 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.
      View in: PubMed   Mentions: 1     Fields:    Translation:HumansAnimalsCells
    12. 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.
      View in: PubMed   Mentions: 3     Fields:    Translation:HumansAnimalsCells
    13. 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.
      View in: PubMed   Mentions: 5     Fields:    Translation:AnimalsCells
    14. 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.
      View in: PubMed   Mentions:    Fields:    
    15. Live Imaging of Organelle Motility in RPE Flatmounts. Adv Exp Med Biol. 2019; 1185:389-393. Umapathy A, Williams DS. PMID: 31884643.
      View in: PubMed   Mentions: 1     Fields:    Translation:HumansCells
    16. 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.
      View in: PubMed   Mentions: 14     Fields:    Translation:HumansCells
    17. 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.
      View in: PubMed   Mentions: 7     Fields:    Translation:HumansAnimalsCells
    18. 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.
      View in: PubMed   Mentions:    Fields:    Translation:AnimalsCells
    19. 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.
      View in: PubMed   Mentions: 3     Fields:    Translation:AnimalsCells
    20. 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.
      View in: PubMed   Mentions: 12     Fields:    Translation:HumansAnimalsCells
    21. 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.
      View in: PubMed   Mentions: 7     Fields:    Translation:HumansCells
    22. 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.
      View in: PubMed   Mentions: 2     Fields:    Translation:AnimalsCells
    23. 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.
      View in: PubMed   Mentions: 38     Fields:    Translation:AnimalsCells
    24. 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.
      View in: PubMed   Mentions: 21     Fields:    Translation:AnimalsCells
    25. 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.
      View in: PubMed   Mentions: 49     Fields:    Translation:HumansAnimals
    26. 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.
      View in: PubMed   Mentions: 7     Fields:    Translation:HumansAnimalsCells
    27. 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.
      View in: PubMed   Mentions: 19     Fields:    Translation:HumansAnimalsCells
    28. 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.
      View in: PubMed   Mentions: 24     Fields:    Translation:HumansAnimalsCells
    29. 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.
      View in: PubMed   Mentions: 30     Fields:    Translation:HumansAnimalsCells
    30. 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.
      View in: PubMed   Mentions: 23     Fields:    Translation:AnimalsCells
    31. 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.
      View in: PubMed   Mentions: 6     Fields:    Translation:HumansAnimalsCells
    32. 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.
      View in: PubMed   Mentions: 1     Fields:    Translation:HumansAnimalsCells
    33. 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.
      View in: PubMed   Mentions: 6     Fields:    Translation:AnimalsCells
    34. 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.
      View in: PubMed   Mentions: 112     Fields:    Translation:HumansAnimalsCells
    35. 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.
      View in: PubMed   Mentions: 7     Fields:    Translation:HumansAnimalsCells
    36. 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.
      View in: PubMed   Mentions: 53     Fields:    Translation:HumansAnimalsCells
    37. 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.
      View in: PubMed   Mentions: 4     Fields:    Translation:AnimalsCells
    38. 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.
      View in: PubMed   Mentions: 35     Fields:    Translation:HumansAnimalsCells
    39. 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.
      View in: PubMed   Mentions: 68     Fields:    Translation:HumansAnimalsCells
    40. 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.
      View in: PubMed   Mentions: 13     Fields:    Translation:AnimalsCells
    41. Gene therapy strategies for Usher syndrome type 1B. Adv Exp Med Biol. 2012; 723:235-42. Williams DS, Lopes VS. PMID: 22183338.
      View in: PubMed   Mentions: 1     Fields:    Translation:HumansAnimalsCells
    42. 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.
      View in: PubMed   Mentions: 36     Fields:    Translation:AnimalsCells
    43. 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.
      View in: PubMed   Mentions: 22     Fields:    Translation:HumansCells
    44. 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.
      View in: PubMed   Mentions: 20     Fields:    Translation:HumansAnimalsCells
    45. 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.
      View in: PubMed   Mentions: 7     Fields:    Translation:AnimalsCells
    46. 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.
      View in: PubMed   Mentions: 26     Fields:    Translation:AnimalsCells
    47. 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.
      View in: PubMed   Mentions: 25     Fields:    Translation:HumansAnimalsCells
    48. 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.
      View in: PubMed   Mentions: 30     Fields:    Translation:AnimalsCells
    49. 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.
      View in: PubMed   Mentions: 168     Fields:    Translation:HumansAnimalsCells
    50. 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.
      View in: PubMed   Mentions: 71     Fields:    Translation:HumansCells
    51. 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.
      View in: PubMed   Mentions: 107     Fields:    Translation:HumansAnimalsCells
    52. Ciliary transport of opsin. Adv Exp Med Biol. 2010; 664:185-91. Trivedi D, Williams DS. PMID: 20238016; PMCID: PMC2933454.
      View in: PubMed   Mentions: 5     Fields:    Translation:HumansAnimalsCells
    53. 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.
      View in: PubMed   Mentions: 19     Fields:    Translation:AnimalsCells
    54. 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.
      View in: PubMed   Mentions: 50     Fields:    Translation:AnimalsCells
    55. 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.
      View in: PubMed   Mentions: 36     Fields:    Translation:AnimalsCells
    56. 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.
      View in: PubMed   Mentions: 35     Fields:    Translation:AnimalsCells
    57. 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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    58. 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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    59. 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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    60. 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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    61. 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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    62. 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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    63. 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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    64. 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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    65. 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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    66. 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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    67. 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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    68. 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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    69. 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.
      View in: PubMed   Mentions: 17     Fields:    Translation:AnimalsCells
    70. 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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    71. 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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    72. 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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    73. 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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    74. 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.
      View in: PubMed   Mentions: 11     Fields:    Translation:AnimalsCells
    75. 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.
      View in: PubMed   Mentions: 7     Fields:    Translation:HumansAnimalsCells
    76. 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.
      View in: PubMed   Mentions: 11     Fields:    Translation:AnimalsCells
    77. 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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    78. 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.
      View in: PubMed   Mentions: 56     Fields:    Translation:HumansAnimalsCells
    79. 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.
      View in: PubMed   Mentions: 8     Fields:    Translation:AnimalsCells
    80. 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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    81. 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.
      View in: PubMed   Mentions: 428     Fields:    Translation:HumansAnimalsCells
    82. 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.
      View in: PubMed   Mentions: 154     Fields:    Translation:HumansCells
    83. 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.
      View in: PubMed   Mentions: 56     Fields:    Translation:AnimalsCells
    84. 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.
      View in: PubMed   Mentions: 21     Fields:    Translation:AnimalsCells
    85. 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.
      View in: PubMed   Mentions: 185     Fields:    Translation:HumansAnimalsCells
    86. 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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    87. 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.
      View in: PubMed   Mentions: 14     Fields:    Translation:Animals
    88. 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.
      View in: PubMed   Mentions: 7     Fields:    Translation:HumansAnimalsCells
    89. 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.
      View in: PubMed   Mentions: 109     Fields:    Translation:AnimalsCells
    90. 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.
      View in: PubMed   Mentions: 105     Fields:    Translation:AnimalsCells
    91. Isolation and culture of primary mouse retinal pigmented epithelial cells. Adv Exp Med Biol. 2003; 533:347-52. Gibbs D, Williams DS. PMID: 15180284.
      View in: PubMed   Mentions: 18     Fields:    Translation:AnimalsCells
    92. 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.
      View in: PubMed   Mentions: 14     Fields:    Translation:Animals
    93. 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.
      View in: PubMed   Mentions:    Fields:    Translation:AnimalsCells
    94. Transport to the photoreceptor outer segment by myosin VIIa and kinesin II. Vision Res. 2002 Feb; 42(4):455-62. Williams DS. PMID: 11853761.
      View in: PubMed   Mentions: 30     Fields:    Translation:AnimalsCells
    95. 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.
      View in: PubMed   Mentions: 30     Fields:    Translation:AnimalsCells
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