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Stein Eye Institute | Doheny Eye Centers

Photoreceptor - RPE Cell Biology

Photoreceptor - RPE Cell Biology

Photoreceptor - RPE Cell Biology

  • Lab Members
    • Barry L. Burgess
    • Abhishek Chadha, PhD
    • Blake Gilmore
    • Roni Hazim, PhD
    • Nan Hultgren, PhD
    • Antonio Escudero Paniagua, PhD
    • Ankita Umapathy, PhD
    • Stefanie Volland, PhD
    • David S. Williams, PhD
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SELECTED PUBLICATIONS RELATED DIRECTLY TO CURRENT RESEARCH

Links to other publications

SELECTED PUBLICATIONS, 1980 - PRESENT

ALL PUBLICATIONS
  • Chadha A, Volland S, Baliaouri NV, Tran EM, Williams DS: The route of the visual receptor rhodopsin along the cilium. Journal of Cell Science, 132(10), 2019.
    https://www.ncbi.nlm.nih.gov/pubmed/30975916 PMID:  30975916

  • Esteve-Rudd J, Hazim RA, Diemer T, Paniagua AE, Volland S, Umapathy A, Williams DS: Defective phagosome motility and degradation in cell non-autonomous RPE pathogenesis of a dominant macular degeneration.
    Proceedings of the National Academy of Sciences, 115:5468-5473, 2018.
    http://www.ncbi.nlm.nih.gov/pubmed/29735674 PMID:  29735674

  • 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: Differentiation of RPE cells from integration-free iPS cells and their cell biological characterization. Stem Cell Research & Therapy. 8:217, 2017 https://www.ncbi.nlm.nih.gov/pubmed/28969679 PMID: 28969679

  • Volland S, Hughes LC, Kong C, Burgess BL, Linberg KA, Luna G, Zhou ZH, Fisher SK, and Williams DS:
    Three-dimensional organization of nascent rod outer segment disk membranes. 
    Proceedings of the National Academy of Sciences 112(48):14870-14875, 2015.
    http://www.ncbi.nlm.nih.gov/pubmed/26578801 PMID: 26578801

  • Jiang M, Esteve-Rudd J, Lopes VS, Diemer T, Lillo C, Rump A, Williams DS:
    Microtubule motors transport phagosomes in the RPE and lack of KLC1 leads to AMD-like pathogenesis. 
    Journal of Cell Biology 210(4):595-611, 2015. 
    http://www.ncbi.nlm.nih.gov/pubmed/26261180 PMID: 26261180

  • Lopes VS, Boye SE, Louie CM, Boye S, Dyka F, Chiodo V, Fofo H, Hauswirth WW, Williams DS:
    Retinal gene therapy with a large MYO7A cDNA using Adeno-associated virus. 
    Gene Therapy 20:824-833, 2013. 
    http://www.ncbi.nlm.nih.gov/pubmed/23344065 PMID: 23344065

  • Trivedi D, Colin E, Louie CM, Williams DS:
    Live-cell imaging evidence for the ciliary transport of rod photoreceptor opsin by heterotrimeric kinesin-2. 
    Journal of Neuroscience 32:10587-10593, 2012. 
    http://www.ncbi.nlm.nih.gov/pubmed/22855808 PMID: 22855808

  • Lopes VS, Gibbs D, Libby RT, Aleman TS, Welch DL, Lillo C, Jacobson SG, Radu RA, Steel KP, Williams DS:
    The Usher 1B protein, MYO7A, is required for normal localization and function of the visual retinoid cycle enzyme, RPE65. 
    Human Molecular Genetics 20:2560-2570, 2011. 
    http://www.ncbi.nlm.nih.gov/pubmed/21493626 PMID: 21493626

  • Lopes VS, Jimeno D, Khanobdee K, Song X, Chen B, Nusinowitz S, Williams DS:
    Dysfunction of Heterotrimeric Kinesin-2 in Rod Photoreceptor Cells and the Role of Opsin Mislocalization in Rapid Cell Death. 
    Molecular Biology of the Cell 21:4076-4088 (plus front cover), 2010.
    http://www.ncbi.nlm.nih.gov/pubmed/20926680 PMID: 20926680

  • Louie CM, Caridi G, Lopes VS, Brancati F, Kispert A, Lancaster MA, Schlossman AM, Otto EA, Leitges M, Grone HJ, Lopez I, Gudiseva HV, O'Toole JF, Vallespin E, Ayyagari R, Ayuso C, Cremers FP, den Hollander Al, Koenekoop RK, Dallapiccola B, Ghiggeri GM, Hildebrandt F, Valente EM, Williams DS, Gleeson JG:
    AHI1 is required for photoreceptor outer segment development and is a modifier for retinal degeneration in nephronophthisis.
    Nature Genetics 42:175-180, 2010.
    http://www.ncbi.nlm.nih.gov/pubmed/20081859 PMID: 20081859

  • Schwander M, Lopes V, Sczaniecka A, Gibbs D, Lillo C, Delano D, Tarantino LM, Wiltshire T, Williams DS, Müller U:
    A novel allele of myosin VIIa reveals a critical function for the C-terminal FERM domain for melanosome transport in retinal pigment epithelial cells. 
    Journal of Neuroscience 29:15810-15818, 2009.
    http://www.ncbi.nlm.nih.gov/pubmed/20016096 PMID: 20016096

  • Yang Z*, Chen Y, Lillo C, Chien J, Yu Z, Michaelides M, Klein M, Howes KA, Li Y, Kaminoh Y, Chen H, Zhao C, 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*:
    Mutant prominin 1 found in patients with macular degeneration disrupts photoreceptor disk morphogenesis in mice. 
    Journal of Clinical Investigation 118:2908-2916, 2008. 
    *Co-corresponding authors. 
    http://www.ncbi.nlm.nih.gov/pubmed/18654668 PMID: 18654668

  • Hashimoto T, Gibbs D, Lillo C, Azarian SM, Legacki E, Zhang X-M, Yang XJ, Williams DS:
    Lentiviral gene replacement therapy of retinas in a mouse model for Usher syndrome type 1B. 
    Gene Therapy 14:584-594, 2007. 
    http://www.ncbi.nlm.nih.gov/pubmed/17268537 PMID: 17268537


  • Karan G, Lillo C, Yang Z, Cameron J, Locke KG, Zhao Y, Thirumalaichary S, Li C, Birch DG, Vollmer-Sanar H, Williams DS*, Zhang K*:
    Lipofuscin accumulation, abnormal electrophysiology and photoreceptor degeneration in mutant ELOVL4 transgenic mice: a model for macular degeneration. 
    Proceedings of the National Academy Sciences 102:4164-4169, 2005. 
    *Co-corresponding authors
    http://www.ncbi.nlm.nih.gov/pubmed/15749821 PMID: 15749821

  • Gibbs D, Azarian SA, Lillo C, Klomp AEM, Kitamoto J, Steel KP, Libby RT, Williams DS:
    Role of myosin VIIa and Rab27a in the motility and localization of RPE melanosomes. 
    Journal of Cell Science 117:6473-6483, 2004.
    http://www.ncbi.nlm.nih.gov/pubmed/15572405 PMID: 15572405

  • Gibbs D, Kitamoto J, Williams DS:
    Abnormal phagocytosis by retinal pigmented epithelium that lacks myosin VIIa, the Usher syndrome 1B protein.
    Proceedings of the National Academy Sciences 100:6481-6486, 2003.
    http://www.ncbi.nlm.nih.gov/pubmed/12743369 PMID: 12743369

  • Marszalek JR, Liu X, Roberts E, Chui D, Marth J, Williams DS, Goldstein LSB:
    Genetic evidence for a kinesin-II mediated pathway for opsin and arrestin transport in mammalian photoreceptors. 
    Cell 102:175-187, 2000.
    http://www.ncbi.nlm.nih.gov/pubmed/10943838 PMID: 10943838

  • Liu X, Udovichenko, IP, Brown SDM, Steel KP, Williams DS: 
    Myosin VIIa participates in opsin transport through the photoreceptor cilium. 
    Journal of Neuroscience 19:6267-6274, 1999. 
    http://www.ncbi.nlm.nih.gov/pubmed/10414956 PMID: 10414956

  • Liu X, Ondek B, Williams DS: 
    Mutant myosin VIIa causes defective melanosome distribution in the RPE of shaker-1 mice.
    Nature Genetics 19:117-118, 1998. 
    http://www.ncbi.nlm.nih.gov/pubmed/9620764 PMID: 9620764

  • Arikawa K, Molday LL, Molday RS, Williams DS:
    Localization of peripherin/rds in the disk membranes of cone and rod photoreceptors: relationship to disk membrane morphogenesis and retinal degeneration. 
    Journal of Cell Biology 116:659-667, 1992.
    http://www.ncbi.nlm.nih.gov/pubmed/1730772 PMID: 1730772
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