All Publications

ALL PUBLICATIONS (140+ journal articles plus book chapters, etc.)

• 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, and Singh R: A human model of Batten disease shows role of CLN3 in phagocytosis at the photoreceptor-RPE interface. Communications Biology 4:161, 2021. https://www.ncbi.nlm.nih.gov/pubmed/33547385             doi:  10.1038/s42003-021-01682-5

• 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, and Hughes CCW: Slug regulates the Dll4-Notch-VEGFR2 axis to control endothelial cell activation and angiogenesis. Nature Communications. 11:5400, 2020.

  https://www.ncbi.nlm.nih.gov/pubmed/33106502             doi.org/10.1038/s41467-020-18633-z

• Segawa M, Wolf DM, Hultgren NW, Williams DS, van der Bliek AM, Shackelford DB, Liesa M, Shirihai OS: Quantification of cristae architecture reveals time-dependent characteristics of individual mitochondria.  Life Science Alliance, 2020.

  https://pubmed.ncbi.nlm.nih.gov/32499316/                    PMID: 32499316           doi: 10.26508/lsa.201900620

• Jiang M, Paniagua AE, Volland S, Wang H, Balaji A, Li DG, Lopes VS, Burgess BL, Williams DS: Microtubule motor transport in the delivery of melanosomes to the actin-rich apical domain of the retinal pigment epithelium. Journal of Cell Science 133, 2020.

  https://www.ncbi.nlm.nih.gov/pubmed/32661088             doi: 10.1242/jcs.242214

• Lakkaraju, A., Umapathy, A., Tan, L.X., Daniele, L., Philp, N.J., Boesze-Battaglia, K., Williams, DS: The cell biology of the retinal pigment epithelium. Progress in Retina and Eye Research, 2020.

   https://www.ncbi.nlm.nih.gov/pubmed/32105772            doi.org/10.1016/j.preteyeres.2020.100846

• Milstein ML, Cavanaugh B, Roussey NM, Volland S, Williams DS, Goldberg AFX: Multistep peripherin-2/rds self-assembly drives membrane curvature for outer segment disk architecture and photoreceptor viability.  Proceedings of the National Academy of Sciences, 2020.
  
  

• Lakkaraju, A., Umapathy, A., Tan, L.X., Daniele, L., Philp, N.J., Boesze-Battaglia, K., Williams, D.S: The cell biology of the retinal pigment epithelium. Progress in Retina and Eye Research, 2020.

  https://www.ncbi.nlm.nih.gov/pubmed/32105772            
  doi: 10.1016/j.preteyeres.2020.100846

• Segawa M, Wolf DM, Hultgren NW, Williams DS, van der Bliek AM, Shackelford DB, Liesa M, Shirihai OS: Quantification of cristae architecture reveals time-dependent characteristics of individual mitochondria.  Life Science Alliance, 2020.

  https://pubmed.ncbi.nlm.nih.gov/32499316/                   PMID: 32499316         
  doi: 10.26508/lsa.201900620

• Daniele LL, Caughey J, Volland S, Sharp RC, Dhingra A, Williams DS, Philp NJ, and Boesze-Battaglia K: Peroxisome turnover and diurnal modulation of antioxidant activity in retinal pigment epithelia utilizes microtubule-associated protein 1 light chain 3B. American Journal of Physiology-Cell Physiology, 317:C1194-C1204, 2019.
  https://www.ncbi.nlm.nih.gov/pubmed/31577510
  
  
• 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
  
  
• Hazim RA, Volland S, Yen A, Burgess BL, Williams DS: Rapid differentiation of the human RPE cell line, ARPE-19, induced by nicotinamide.  
  Experimental Eye Research, 179:18-24, 2019
  https://www.ncbi.nlm.nih.gov/pubmed/30336127 PMID: 30336127
  
  

• Volland S., Williams D.S. (2018) Preservation of Photoreceptor Nanostructure for Electron Tomography Using Transcardiac Perfusion Followed by High-Pressure Freezing and Freeze-Substitution. In:
  Ash J., Anderson R., LaVail M., Bowes Rickman C., Hollyfield J., Grimm C. (eds)
  Retinal Degenerative Diseases. Advances in Experimental Medicine and Biology, vol 1074. Springer, Cham
  https://link.springer.com/chapter/10.1007%2F978-3-319-75402-4_73#citeas
  

• 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
  
  
• Galloway CA, Dalvi S, Hung SSC, MacDonald LA, Latchney LR, Wong RCB, Guymer RH, Mackey DA, Williams DS, Chung MM, Gamm DM, Pebay A, Hewitt AW, Singh R: Drusen in patient-derived hiPSC-RPE models of macular dystrophies. Proceedings of the National Academy of Sciences. 114:E8214-E8223, 2017
  https://www.ncbi.nlm.nih.gov/pubmed/28878022 PMID: 28878022
  
  
• Orme MH, Liccardi G, Moderau N, Feltham R, Wicky-John S, Tenev T, Aram L, Wilson R, Bianchi K, Morris O, Monteiro Domingues C, Robertson D, Tare M, Wepf A, Williams D, Bergmann A, Gstaiger M, Arama E, Ribeiro PS, Meier P: The unconventional myosin CRINKLED and its mammalian orthologue MYO7A regulate caspases in their signaling roles. Nature Communications, 7:10972 (1-12), 2016.
  http://www.ncbi.nlm.nih.gov/pubmed/26960254
   
• Goldberg AG, Moritz OL, Williams DS:  Molecular basis for photoreceptor outer segment architecture.  Progress in Retinal and Eye Research, 55:52-81, 2016.
  http://www.ncbi.nlm.nih.gov/pubmed/27260426
   
• Williams DS, Chadha A, Hazim R, Gibbs D:  Gene Therapy approaches for prevention of retinal degeneration in Usher syndrome.  Gene Therapy, 24:68-71.
  https://www.ncbi.nlm.nih.gov/pubmed/28054582

   

• 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
  
  
• Volland S, Esteve-Rudd J, Hoo J, Yee C, Williams DS: A comparison of some organizational characteristics of the mouse central retina and the human macula. PLos One, 10:e0125631, 2015.
  http://www.ncbi.nlm.nih.gov/pubmed/25923208 PMID: 25923208
  
  
• 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: Spata7 is a retinal ciliopathy gene critical for correct RPGRIP1 localization and protein trafficking in the retina. Human Molecular Genetics, 24:1584-1601, 2015.
  http://www.ncbi.nlm.nih.gov/pubmed/25398945 PMID: 25398945
  
  
• Frost LS, Lopes VS, Bragin A, Reyes-Reveles J, Brancato J, Cohen A, Mitchell CH, Williams DS, Boesze-Battaglia K: The contribution of melanoregulin to microtubule-associated protein 1 light chain 3 (LC3) associated phagocytosis in retinal pigment epithelium. Molecular Neurobiology epub, 2014.
  http://www.ncbi.nlm.nih.gov/pubmed/25301234 PMID: 25301234
  
  
• 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: Mutations in EMP2 cause childhood-onset nephrotic syndrome. American Journal of Human Genetics 94:884-90, 2014.
  http://www.ncbi.nlm.nih.gov/pubmed/24814193 PMID: 24814193
  
  
• Crouse JA, Lopes VS, SanAgustin JT, Keady BT, Williams DS, Pazour GJ: Distinct functions for IFT140 and IFT20 in opsin transport. Cytoskeleton 71:302-310, 2014.
  http://www.ncbi.nlm.nih.gov/pubmed/24619649 PMID: 24619649
  
  
• 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: UV irradiation accelerates amyloid precursor protein (APP) processing and disrupts APP axonal transport. Journal of Neuroscience 34:3320-39, 2014.
  http://www.ncbi.nlm.nih.gov/pubmed/24573290 PMID: 24573290
  
  
• Mei H, Gonzalez S, Nakatsu MN, Baclagon ER, Lopes VS, Williams DS, Deng SX: A Three-dimensional culture method to expand limbal stem/progenitor cells. Tissue engineering. Part C, Methods, 2014.
  http://www.ncbi.nlm.nih.gov/pubmed/24047104 PMID: 24047104
  
  
• 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. (2013) ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption. Journal of Clinical Investigation 123(12):5179-89, 2013.
  http://www.ncbi.nlm.nih.gov/pubmed/24270420 PMID: 24270420
  
  
• 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, Kohler 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: ZMYND10 Is Mutated in Primary Ciliary Dyskinesia and Interacts with LRRC6. American Journal of Human Genetics 93:336-345, 2013.
  http://www.ncbi.nlm.nih.gov/pubmed/23891469 PMID: 23891469
  
  
• Frost LS, Lopes VS, Stefano FP, Bragin A, Williams DS, Mitchell CH, Boesze-Battaglia K: Loss of Melanoregulin (MREG) enhances Cathepsin-D secretion by the Retinal Pigment Epithelium (RPE). Visual Neuroscience 30:55-64. 2013.
  http://www.ncbi.nlm.nih.gov/pubmed/23611523 PMID: 23611523
  
  
• 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
  
  
• Strong R, Miller RA, Astle CM, Baur JA, de Cabo R, Fernandez E, Guo W, Javors M, Kirkland JL, Nelson JF, Sinclair DA, Teter B, Williams D, Zaveri N, Nadon NL, Harrison DE: Evaluation of resveratrol, green tea extract, curcumin, oxaloacetic acid, and medium-chain triglyceride oil on life span of genetically heterogeneous mice. Journal of Gerontology A Biological Sciences and Medical Sciences 68:6-16, 2013.
  http://www.ncbi.nlm.nih.gov/pubmed/22451473 PMID: 22451473
  
  
• 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
  
  
• Da Cruz S, Parone PA, Lopes VS, Lillo C, McAlonis-Downes M, Lee SK, Vetto AP, Petrosyan S, Marsala MS, Murphy AN, Williams DS, Spiegelman BM, Cleveland DW: Elevated PGC-1α activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS. Cell Metabolism 15:778-786, 2012.
  http://www.ncbi.nlm.nih.gov/pubmed/22560226 PMID: 22560226
  
  
• Engelhardt M, Tosha C, Lopes VS, Chen B, Nguyen L, Nusinowitz S, Williams DS: Functional and morphological analysis of the subretinal injection of RPE cells. Visual Neuroscience 29:83-93 (plus front cover), 2012.
  http://www.ncbi.nlm.nih.gov/pubmed/22391151 PMID: 22391151
  
  
• 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: Essential role of ELOVL4 in very long chain fatty acid synthesis and retinal function. Journal of Biological Chemistry 287:11469-11480, 2012.
  http://www.ncbi.nlm.nih.gov/pubmed/22199362 PMID: 22199362
  
  
• Williams DS, Lopes VS: The many different cellular functions of MYO7A in the retina. Biochemical Society transactions 39:1207-10, 2011.
  http://www.ncbi.nlm.nih.gov/pubmed/21936790 PMID: 21936790
  
  
• 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: Retinal Disease Course in Usher Syndrome 1B due to MYO7A Mutations. Investigative Ophthalmology & Visual Science, 52:7924-7936, 2011.
  http://www.ncbi.nlm.nih.gov/pubmed/21873662 PMID: 21873662
  
  
• Phillips JB, Blanco-Sanchez B, Lentz JJ, Tallafuss A, Khanobdee K, Sampath S, Jacobs ZG, Han PF, Mishra M, Williams DS, Keats BJ, Washbourne P, Westerfield M: Harmonin (Ush1c) is required in zebrafish Muller glial cells for photoreceptor synaptic development and function. Disease Models & Mechanisms 4:786-800, 2011.
  http://www.ncbi.nlm.nih.gov/pubmed/21757509 PMID: 21757509
  
  
• 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
  
  
• 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, Obermuller 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, Nurnberg G, Nurnberg P, Pierce EA, Jackson PK, Antignac C, Saunier S, Roepman R, Dollfus H, Khanna H, Hildebrandt F: Candidate exome capture identifies mutation of SDCCAG8 as the cause of a retinal-renal ciliopathy. Nature Genetics 42:840-850, 2010.
  http://www.ncbi.nlm.nih.gov/pubmed/20835237 PMID: 20835237
  
  
• Liao JL,Yu J, Huang K, Hu J, Diemer T, Ma Z, Dvash T, Yang XJ, Travis GH, Williams DS, Bok D, Fan G: Molecular signature of primary retinal pigment epithelium and stem-cell-derived RPE cells. Human Molecular Genetics 19:4229-4238, 2010.
  http://www.ncbi.nlm.nih.gov/pubmed/20709808 PMID: 20709808
  
  
• 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 AI, 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
  
  
• Gibbs D, Diemer T, Khanobdee K, Hu J, Bok D, Williams DS: Function of MYO7A in the human RPE and the validity of shaker1 mice as a model for Usher syndrome 1B. Investigative Ophthalmology and Visual Science 51:1130-1135, 2010.
  http://www.ncbi.nlm.nih.gov/pubmed/19643958 PMID: 19643958
  
  
• 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
  
  
• Abe N, Almenar-Queralt A, Lillo C, Shen Z, Lozach J, Briggs SP, Williams DS, Goldstein LS, Cavalli V: Sunday Driver interacts with two distinct classes of axonal organelles. Journal of Biological Chemistry, 284:34628-34639, 2009.
  http://www.ncbi.nlm.nih.gov/pubmed/19801628 PMID: 19801628
  
  
• Avasthi P, Watt CB, Williams DS, Le YZ, Li S, Chen C-K, Marc RE, Frederick JM, Baehr W: Trafficking of membrane proteins to cone but not rod outer segments is dependent on heterotrimeric kinesin-II. Journal of Neuroscience 29:14287-14298, 2009.
  http://www.ncbi.nlm.nih.gov/pubmed/19906976 PMID: 19906976
  
  
• Williams DS, Cash A, Hamadani L, Diemer T: Oxaloacetate supplementation increases lifespan in Caenorhabditis elegans through an AMPK/FOXO-dependent pathway. Aging Cell, 8:765-768, 2009.
  http://www.ncbi.nlm.nih.gov/pubmed/19793063 PMID: 19793063
  
  
• Grillet N, Xong W, Reynolds A, Kazmierczak P, Sato T, Lillo C, Dumont RA, Hintermann E, Sczaniecka A, Schwander M, Williams DS, Kachar B, Gillespie PG, Mueller U: Harmonin mutations cause mechanotransduction defects in cochlear hair cells. Neuron 62:375-387, 2009.
  http://www.ncbi.nlm.nih.gov/pubmed/19447093 PMID: 19447093
  
  
• Falzone TL, Stokin GB, Lillo C, Rodrigues EM, Westerman EL, Williams DS, Goldstein LSB: Axonal stress kinase activation and tau misbehavior induced by kinesin-1 transport defects. Journal of Neuroscience 29:5758-5767, 2009.
  http://www.ncbi.nlm.nih.gov/pubmed/19420244 PMID: 19420244
  
  
• Gibbs D, Cideciyan AV, Jacobson SG, Williams DS: Retinal pigment epithelium defects in humans and mice with mutations in MYO7A: imaging melanosome-specific autofluorescence. Investigative Ophthalmology and Visual Science 50:4386-4393, 2009.
  http://www.ncbi.nlm.nih.gov/pubmed/19324852 PMID: 19324852
  
  
• Williams DS, Aleman TS, Lillo C, Lopes VS, Hughes LC, Stone EM, Jacobson SG: Harmonin in the murine retina and the retinal phenotypes of Ush1c-mutant mice and human USH1C. Investigative Ophthalmology and Visual Science 50:3881-3889, 2009.
  http://www.ncbi.nlm.nih.gov/pubmed/19324851 PMID: 19324851
  
  
• Damek-Poprawa M, Diemer T, Lopes VS, Lillo C, Harper, DC, Marks MS, Wu Y, Sparrow JR, Rachel R, Williams DS, Boesze-Battaglia K: Melanoregulin (MREG) modulates lysosome function in pigment epithelial cells. Journal of Biological Chemistry 284:10877-10889, 2009.
  http://www.ncbi.nlm.nih.gov/pubmed/19240024 PMID: 19240024
  
  
• Stokin GB, Almenar-Queralt A, Gunawardena S, Rodrigues EM, Falzone T, Kim J, Lillo C, Mount SL, Roberts EA, McGowan E, Williams DS, Goldstein LSB: Amyloid precursor protein-induced axonopathies are independent of amyloid-beta peptides. Human Molecular Genetics 17:3474-3486, 2008.
  http://www.ncbi.nlm.nih.gov/pubmed/18694898 PMID: 18694898
  
  
• 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
  
  
• Jacobson SG, Cideciyan AV, Aleman TS, Sumaroka A, Roman AJ, Gardner LM, Prosser HM, Mishra M, Bech-Hansen T, Herrera W, Schwartz SB, Liu X-Z, Kimberling WJ, Steel KP, Williams DS. Usher syndromes due to MYO7A, PCDH15, USH2A or GPR98 mutations share retinal disease mechanism. Human Molecular Genetics 17:2405-2415, 2008.
  http://www.ncbi.nlm.nih.gov/pubmed/18463160 PMID: 18463160
  
  
• Allocca M, Doria M, Petrillo M, Colella P, Garcia-Hoyos M, Gibbs D, Kim SR, Maguire A, Rex TS, Vicino U, Cutillo L, Sparrow JR, Williams DS, Bennett J, Auricchio A: Serotype-dependent packaging of large genes in adeno-associated viral vectors results in effective gene delivery in mice. Journal of Clinical Investigation 118:1955-1964, 2008.
  http://www.ncbi.nlm.nih.gov/pubmed/18414684 PMID: 18414684
  
  
• Feathers KL, Lyubarsky AL, Khan NW, Teofilo K, Swaroop A, Williams DS, Pugh EN, Thompson DA: Nrl-knockout mice deficient in Rpe65 fail to synthesize 11-cis retinal and cone outer segments. Investigative Ophthalmology and Visual Science 49:1126-1135, 2008.
  http://www.ncbi.nlm.nih.gov/pubmed/18326740 PMID: 18326740
  
  
• Williams DS: Usher syndrome: animal models, retinal function of Usher proteins, and prospects for gene therapy. Vision Research 48:433-441, 2008.
  http://www.ncbi.nlm.nih.gov/pubmed/17936325 PMID: 17936325
  
  
• Klomp AE, Teofilo K, Legacki E, Williams DS: Analysis of the linkage of MYRIP and MYO7A to melanosomes by RAB27A in retinal pigment epithelial cells. Cell Motility and the Cytoskeleton 64:474-487 (plus front cover), 2007.
  http://www.ncbi.nlm.nih.gov/pubmed/17352418 PMID: 17352418
  
  
• 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
  
  
• Boesze-Battaglia K, Song H, Sokolov M, Lillo C, Pankoski-Walker L, Gretzula C, Gallagher B, Rachel RA, Jenkins NA, Copeland NG, Morris F, Yeagle P, Williams DS, Damek-Poprawa M: The tetraspanin protein, peripherin-2, complexes with melanoregulin, a putative membrane fusion regulator. Biochemistry 46:1256-1272, 2007.
  http://www.ncbi.nlm.nih.gov/pubmed/17260955 PMID: 17260955
  
  
• Schubert D, Williams DS: ‘Cisgenic’ as a product designation. Nature Biotechnology 24:1327-1329, 2006.
  http://www.ncbi.nlm.nih.gov/pubmed/17093469 PMID: 17093469
  
  
• Jimeno D, Feiner L, Lillo C, Teofilo K, Goldstein LSB, Pierce E, Williams DS: Analysis of kinesin-2 function in photoreceptor cells using synchronous Cre-loxP knockout of Kif3a with RHO-Cre. Investigative Ophthalmology and Visual Science 47:5039-5046, 2006.
  http://www.ncbi.nlm.nih.gov/pubmed/17065525 PMID: 17065525
  
  
• 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: 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. Human Molecular Genetics 15:1847-1857, 2006.
  http://www.ncbi.nlm.nih.gov/pubmed/16632484 PMID: 16632484
  
  
• 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 M, Neuhaus T, Parisi MA, Glass IA, Petry M, Kispert A, Zou Y, Gloy J, Ganner A, Walz G, Zhu X, Goldman D, Nurnberg P, Swaroop A, Leroux MR, Hildebrandt F: The centrosomal protein, nephrocystin-6, is mutated in Joubert syndrome and activates transcription factor ATF4. Nature Genetics, 38:674-681, 2006.
  http://www.ncbi.nlm.nih.gov/pubmed/16682973 PMID: 16682973
  
  
• Senften M, Schwander M, Kazmierczak P, Lillo C, Shin J-B, Hasson T, Géléoc GSG, Gillespie P, Williams DS, Holt J, Müller U: Physical and functional interaction between protocadherin 15 and myosin VIIa in mechanosensory hair cells. Journal of Neuroscience 26:2060-2071, 2006.
  http://www.ncbi.nlm.nih.gov/pubmed/16481439 PMID: 16481439
  
  
• Azarian SM, McLeod I, Lillo C, Gibbs D, Yates JR, Williams DS: Proteomic analysis of mature melanosomes from the retinal pigmented epithelium. Journal of Proteome Research 5:521-529, 2006.
  http://www.ncbi.nlm.nih.gov/pubmed/16512666 PMID: 16512666
  
  
• Jimeno D, Lillo C, Roberts EA, Goldstein LSB, Williams DS: Kinesin-2 and photoreceptor cell death: requirement of motor subunits. Experimental Eye Research 82:351-353, 2006.
  http://www.ncbi.nlm.nih.gov/pubmed/16337628 PMID: 16337628
  
  
• Zhu X, Wu K, Rife L, Cawley NX, Brown B, Adams T, Teofilo K, Lillo C, Williams DS, Loh YP, Craft CM: Carboxypeptidase E is required for normal synaptic transmission from photoreceptors to the inner retina. Journal of Neurochemistry 95:1351-1362, 2005.
  http://www.ncbi.nlm.nih.gov/pubmed/16219026 PMID: 16219026
  
  
• Khanna H, Hurd TW, Lillo C, Shu X, Parapuram SK, He S, Akimoto M, Wright AF, Margolis B, Williams DS, Swaroop A: RPGR-ORF15, which is mutated in retinitis pigmentosa, associates with SMC1, SMC3, and microtubule transport proteins. Journal of Biological Chemistry 280:33580-33587, 2005.
  http://www.ncbi.nlm.nih.gov/pubmed/16043481 PMID: 16043481
  
  
• Kitamoto J, Libby RT, Gibbs D, Steel KP, Williams DS: Myosin VI is required for normal retinal function. Experimental Eye Research 81:116-120, 2005.
  http://www.ncbi.nlm.nih.gov/pubmed/15978262 PMID: 15978262
  
  
• 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
  
  
• Stokin GB, Lillo C, Falzone T, Brusch RG, Rockenstein E, Mount S, Raman R, Davies P, Masliah E, Williams DS, Goldstein LSB: Axonpathy and transport deficits in the pathogenesis of Alzheimer’s disease. Science 307:1282-1288, 2005.
  http://www.ncbi.nlm.nih.gov/pubmed/15731448 PMID: 15731448
  
  
• 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, Couck P, De Paepe A, Reinhardt R, Klages S, Ysuda M, Kawakami I, Kusakabe T, Omran H, ImmA, Tippens M, Raymond PA, Hill J, Beales P, He S, Kispert A, Margolis B, Williams DS, Swaroop A, Hildebrandt F: Nephrocystin-5, a ciliary IQ domain protein, is mutated in Senior-Loken syndrome and interacts with RPGR and calmodulin. Nature Genetics 37:282-288, 2005.
  http://www.ncbi.nlm.nih.gov/pubmed/15723066 PMID: 15723066
  
  
• Daniele L, Lillo C, Lyubarsky A, Philp N, Mears AJ, Swaroop A, Williams DS, Pugh E: Cone-like morphological, molecular and functional features of the photoreceptors of the Nrl-/- knockout mouse. Investigative Ophthalmology and Visual Science 46:2156-2167, 2005.
  http://www.ncbi.nlm.nih.gov/pubmed/15914637 PMID: 15914637
  
  
• 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
  
  
• Libby RT, Lillo C, Kitamoto J, Williams DS, Steel KP: Myosin Va is required for normal photoreceptor synaptic activity. Journal of Cell Science 117:4509-4515, 2004.
  http://www.ncbi.nlm.nih.gov/pubmed/15316067 PMID: 15316067
  
  
• Liu J, Lillo C, Jonsson PA, Ward CM, Subramaniam JR, Rothstein JD, Marklund S, Andersen PM, Brännström T, Wong PC, Williams DS, Cleveland DW: Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron 43:5-17, 2004.
  http://www.ncbi.nlm.nih.gov/pubmed/15233913 PMID: 15233913
  
  
• Siemens J, Lillo C, Dumont RA, Williams DS, Gillespie PG, Müller U: Cadherin 23 is a component of the tip link in hair cell stereocilia. Nature 428:950-955, 2004.
  http://www.ncbi.nlm.nih.gov/pubmed/15057245 PMID: 15057245
  
  
• Libby RT, Kitamoto J, Holme RH, Williams DS, Steel KP: Cdh23 mutations in the mouse associated with retinal dysfunction but not retinal degeneration. Experimental Eye Research 77:731-739, 2003.
  http://www.ncbi.nlm.nih.gov/pubmed/14609561 PMID: 14609561
  
  
• Adams RA, Liu X, Williams DS, Newton AC: Differential spatial and temporal phosphorylation of the visual receptor, rhodopsin, at its two primary phosphorylation sites in mice exposed to light. Biochemical Journal 374:537-543, 2003.
  http://www.ncbi.nlm.nih.gov/pubmed/12809555 PMID: 12809555
  
  
• 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
  
  
• Xia C, Roberts EA, Her L, Liu X, Williams DS, Cleveland, DW, Goldstein LS: Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A. Journal of Cell Biology, 161:55-66, 2003.
  http://www.ncbi.nlm.nih.gov/pubmed/12682084 PMID: 12682084
  
  
• Udovichenko IP, Gibbs D, Williams DS: Actin-based motor properties of native myosin VIIa. Journal of Cell Science, 115:445-450, 2002.
  http://www.ncbi.nlm.nih.gov/pubmed/11839794 PMID: 11839794
  
  
• Williams DS: Transport to the photoreceptor outer segment by myosin VIIa and kinesin II. Vision Research, 42:455-462, 2002.
  http://www.ncbi.nlm.nih.gov/pubmed/11853761 PMID: 11853761
  
  
• Liu X, Williams DS: Coincident onset of expression of myosin VIIa and opsin in the cilium of the developing photoreceptor cell. Experimental Eye Research 72:351-355, 2001.
  http://www.ncbi.nlm.nih.gov/pubmed/11180984 PMID: 11180984
  
  
• 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
  
  
• Richardson GP, Forge A, Kros CJ, Marcotti W, Becker D, Williams DS, Thorpe J, Fleming J, Brown SDM, Steel KP: A missense mutation in myosin VIIA prevents aminoglycoside accumulation in cochlear hair cells. Annals of the New York Academy of Sciences 884:110-124, 1999.
  http://www.ncbi.nlm.nih.gov/pubmed/10842588 PMID: 10842588
  
  
• Wolfrum U, Liu X, Schmitt A, Udovichenko IP, Williams DS: Myosin VIIa as a common component of cilia and microvilli. Cell Motility and the Cytoskeleton 40:261-271, 1998.
  http://www.ncbi.nlm.nih.gov/pubmed/9678669 PMID: 9678669
  
  
• 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
  
  
• Udovichenko IP, Newton AC, Williams DS: Regulation of the phosphorylation state of rhodopsin by dopamine. Journal of Biological Chemistry 273:7181-7184, 1998.
  http://www.ncbi.nlm.nih.gov/pubmed/9516406 PMID: 9516406
  
  
• Liu X, Vansant G, Udovichenko IP, Wolfrum U, Williams DS: Myosin VIIa, the product of the Usher 1B syndrome gene, is concentrated in the connecting cilia of rod and cone photoreceptor cells. Cell Motility and the Cytoskeleton 37:240-252 (plus front cover), 1997.
  http://www.ncbi.nlm.nih.gov/pubmed/9227854 PMID: 9227854
  
  
• Williams DS, Liu X, Schlamp CS, Ondek B, Jaken S, and Newton AC: Characterization of protein kinase C in photoreceptor outer segments. Journal of Neurochemistry 69:1693-1702, 1997.
  http://www.ncbi.nlm.nih.gov/pubmed/9326298 PMID: 9326298
  
  
• Boesze-Battaglia K, Kong F, Lamba OP, Stefano FP, Williams DS: Purification and light-dependent phosphorylation of a candidate fusion protein, the photoreceptor cell peripherin/rds. Biochemistry 36:6835-6846, 1997.
  http://www.ncbi.nlm.nih.gov/pubmed/9184167 PMID: 9184167
  
  
• Greene NM, Williams DS, Newton AC: Identification of protein kinase C phosphorylation sites on bovine rhodopsin. Journal of Biological Chemistry 272:10341-10344, 1997.
  http://www.ncbi.nlm.nih.gov/pubmed/9099669 PMID: 9099669
  
  
• Udovichenko IP, Newton AC, Williams DS: Contribution of protein kinase C to the phosphorylation of rhodopsin in intact retinas. Journal of Biological Chemistry 272:7952-7976, 1997.
  http://www.ncbi.nlm.nih.gov/pubmed/9065465 PMID: 9065465
  
  
• Liu X, Wu, T-H, Stowe, S, Atsuko M, Arikawa K, Naash M, Williams DS: Defective phototransductive disk membrane morphogenesis in transgenic mice expressing rhodopsin with a mutated N-terminal domain. Journal of Cell Science 110:2589-2597, 1997.
  http://www.ncbi.nlm.nih.gov/pubmed/9372448 PMID: 9372448
  
  
• Schlamp CS, Williams DS: Myosin V in the retina: localization in the rod photoreceptor synapse. Experimental Eye Research 63:613-619, 1996.
  http://www.ncbi.nlm.nih.gov/pubmed/9068368 PMID: 9068368
  
  
• Hicks JL, Liu X, Williams DS: The role of the ninaC proteins in photoreceptor cell structure: ultrastructure of ninaC deletion mutants and binding to actin filaments. Cell Motility and the Cytoskeleton 35:367-379 (plus front cover), 1996.
  http://www.ncbi.nlm.nih.gov/pubmed/8956007 PMID: 8956007
  
  
• Hallett MA, Delaat JL, Arikawa K, Schlamp CL, Kong F, Williams DS: Distribution of guanylate cyclase within rod photoreceptor outer segments. Journal of Cell Science 109:1803-1812, 1996.
  http://www.ncbi.nlm.nih.gov/pubmed/8832403 PMID: 8832403
  
  
• Azarian SM, King AJ, Hallett MA, Williams DS: Selective proteolysis of arrestin by calpain: molecular characterization and its effect on rhodopsin dephosphorylation. Journal of Biological Chemistry 270:24375-24384, 1995.
  http://www.ncbi.nlm.nih.gov/pubmed/7592650 PMID: 7592650
  
  
• Greene NM, Williams DS, Newton AC: Kinetics and localization of the phosphorylation of rhodopsin by protein kinase C. Journal of Biological Chemistry 270:6710-6717, 1995.
  http://www.ncbi.nlm.nih.gov/pubmed/7896814 PMID: 7896814
  
  
• Azarian SM, Williams DS: Calpain activity in the retinas of normal and RCS rats. Current Eye Research 14:731-735, 1995.
  http://www.ncbi.nlm.nih.gov/pubmed/8529410 PMID: 8529410
  
  
• Williams DS: The dynamics of cytosolic calcium in photoreceptor cells. Bioessays 17: 282-286, 1995.
  http://www.ncbi.nlm.nih.gov/pubmed/7741721 PMID: 7741721
  
  
• Williams DS, Park S, Schlamp, CL, Newton AC: Protein kinase C association with the retinal cytoskeleton and phosphorylation of vimentin. Experimental Eye Research 58:747-759, 1994.
  http://www.ncbi.nlm.nih.gov/pubmed/7925714 PMID: 7925714
  
  
• Nickells RW, Schlamp CL, Newton AC, Williams DS: Gene expression of the neuropeptide processing enzyme carboxypeptidase E in rat photoreceptor cells. Journal of Neurochemistry 61:1891-1900, 1993.
  http://www.ncbi.nlm.nih.gov/pubmed/7693871 PMID: 7693871
  
  
• Nickells RW, Newton AC, Williams DS: PCR reamplification of small target cDNAs from high-resolution polyacrylamide gels. Amplifications 9:6-7, 1993.
  
  
• Newton AC, Williams DS: Rhodopsin is the major in situ substrate of protein kinase C in rod outer segments of photoreceptors. Journal of Biological Chemistry 268:18181-18186, 1993.
  http://www.ncbi.nlm.nih.gov/pubmed/8349693 PMID: 8349693
  
  
• Newton AC, Williams DS: Does protein kinase C play a role in rhodopsin desensitization? Trends in Biochemical Sciences 18:275-277, 1993.
  http://www.ncbi.nlm.nih.gov/pubmed/8236436 PMID: 8236436
  
  
• Azarian SM, Schlamp CL, Williams DS: Characterization of calpain II in the retina and photoreceptor outer segments. Journal of Cell Science 105:787-798, 1993.
  http://www.ncbi.nlm.nih.gov/pubmed/8408304 PMID: 8408304
  
  
• Arikawa K, Williams DS: Acetylated alpha-tubulin in the connecting cilium of developing rat photoreceptors. Investigative Ophthalmology and Visual Science 34:2145-2149, 1993.
  http://www.ncbi.nlm.nih.gov/pubmed/8491566 PMID: 8491566
  
  
• Porter JA, Hicks JL, Willliams DS, Montell C: Differential localizations of and requirements for the two Drosophila ninaC kinase/myosins in photoreceptor cells. Journal of Cell Biology 116:683-693, 1992.
  http://www.ncbi.nlm.nih.gov/pubmed/1730774 PMID: 1730774
  
  
• Hicks JL, Williams DS: Distribution of the myosin I-like ninaC proteins in the Drosophila retina and ultrastructural analysis of mutant phenotypes. Journal of Cell Science 101:247-254, 1992.
  http://www.ncbi.nlm.nih.gov/pubmed/1569127 PMID: 1569127
  
  
• 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
  
  
• Williams DS, Hallett M, Arikawa K: Association of myosin with the connecting cilium of rod photoreceptors. Journal of Cell Science 103:183-190, 1992.
  http://www.ncbi.nlm.nih.gov/pubmed/1429904 PMID: 1429904
  
  
• Williams DS, Roberts EA: Modification of the daily photoreceptor membrane shedding response in vitro by antioxidants. Investigative Ophthalmology and Visual Science 33:3005-3008, 1992.
  http://www.ncbi.nlm.nih.gov/pubmed/1526748 PMID: 1526748
  
  
• Päällysaho T, Williams DS: Epithelial cell-substrate adhesion in the cornea: localization of actin, talin, integrin and fibronectin. Experimental Eye Research 52:261-267, 1991.
  http://www.ncbi.nlm.nih.gov/pubmed/1901797 PMID: 1901797
  
  
• Newton AC, Williams DS: Involvement of protein kinase C in the phosphorylation of rhodopsin. Journal of Biological Chemistry 266:17725-17728, 1991.
  http://www.ncbi.nlm.nih.gov/pubmed/1917916 PMID: 1917916
  
  
• Arikawa K, Williams DS: Alpha-actinin and actin in the outer retina: a double immunoelectron microscopical study. Cell Motility and the Cytoskeleton 18:15-25 (plus front cover), 1991.
  http://www.ncbi.nlm.nih.gov/pubmed/2004431 PMID: 2004431
  
  
• Williams DS: Actin filaments and photoreceptor membrane turnover. Bioessays 13:171-178, 1991.
  http://www.ncbi.nlm.nih.gov/pubmed/1859395 PMID: 1859395
  
  
• Arikawa K, Hicks JL, Williams DS: Identification of actin filaments in the rhabdomeral microvilli of Drosophila photoreceptors. Journal of Cell Biology 110:1993-1998, 1990.
  http://www.ncbi.nlm.nih.gov/pubmed/2112548 PMID: 2112548
  
  
• Williams DS, Arikawa K, Päällysaho T: Cytoskeletal components of the adherens junctions between the photoreceptors and the supportive Müller cells. Journal of Comparative Neurology 295:155-164, 1990.
  http://www.ncbi.nlm.nih.gov/pubmed/2341633 PMID: 2341633
  
  
• Williams DS, Linberg KA, Fisher SK: Shedding of cone photoreceptor disk membranes in lizard eyecups in vitro. Experimental Eye Research 50:231-234, 1990.
  http://www.ncbi.nlm.nih.gov/pubmed/2311685 PMID: 2311685
  
  
• Arikawa K, Williams DS: Organization of actin filaments and immunocolocalization of alpha-actinin in the connecting cilium of rat photoreceptors. Journal of Comparative Neurology 288:640-646, 1989.
  http://www.ncbi.nlm.nih.gov/pubmed/2808754 PMID: 2808754
  
  
• Williams DS, Shuster TA, Moldrawski MR, Blest AD, Farber DB: Isolation of rod outer segments on Percoll gradients: effect of specific protease inhibition. Experimental Eye Research 49:439-444, 1989.
  http://www.ncbi.nlm.nih.gov/pubmed/2551708 PMID: 2551708
  
  
• Anderson DH, Williams DS, Neitz J, Farris RN, Fliesler SJ: Tunicamycin-induced degeneration in cone photoreceptors. Visual Neuroscience 1:153-158, 1988.
  http://www.ncbi.nlm.nih.gov/pubmed/3154792 PMID: 3154792
  
  
• Williams DS, Linberg KA, Vaughan DK, Fariss RN, Fisher SK: Disruption of microfilament organization and deregulation of disk membrane morphogenesis by cytochalasin D in rod and cone photoreceptors. Journal of Comparative Neurology 272:161-176, 1988. 
  http://www.ncbi.nlm.nih.gov/pubmed/3397406 PMID: 3397406
  
  
• Shuster TS, Walter AE, Williams DS, Farber DB: Identification of a PNA-binding protein from human retina. Experimental Eye Research 45:685-694, 1987.
  http://www.ncbi.nlm.nih.gov/pubmed/3428393 PMID: 3428393
  
  
• Williams DS, Colley NJ, Farber DB: Photoreceptor degeneration in a pure-cone retina: effects of cyclic nucleotides, and inhibitors of phosphodiesterase and protein synthesis. Investigative Ophthalmology and Visual Science 28:1059-1069, 1987.
  http://www.ncbi.nlm.nih.gov/pubmed/2439472 PMID: 2439472
  
  
• Williams DS, Fisher SK: Prevention of the shedding of rod outer segment disks by detachment from the retinal pigment epithelium. Investigative Ophthalmology and Visual Science 28:184-187, 1987.
  http://www.ncbi.nlm.nih.gov/pubmed/3804649 PMID: 3804649
  
  
• Williams DS, Colley NJ, Anderson DH, Farber DB, Fisher SK: In vitro maintenance of a pure-cone retina. Investigative Ophthalmology and Visual Science 27:666-673, 1986.
  http://www.ncbi.nlm.nih.gov/pubmed/3516917 PMID: 3516917
  
  
• Williams DS, Wilson C, Linberg K, Fisher SK: Effects of low sodium, ouabain, and strophanthidin on the shedding of rod outer segment discs. Journal of Comparative Physiology A 155:763-770, 1984.
  http://link.springer.com/content/pdf/10.1007/BF00611593.pdf
  
  
• Williams DS: Changes in photoreceptor performance associated with the daily turnover of photoreceptor membrane in locusts. Journal of Comparative Physiology A 150:509-519, 1983.
  http://link.springer.com/content/pdf/10.1007/BF00609577.pdf
  
  
• Blest AD, Stowe S, Eddey W, Williams DS: The local deletion of a microvillar cytoskeleton from photoreceptors of tipulid flies during membrane turnover. Proceedings of the Royal Society of London B 215:469-489, 1982.
  http://www.ncbi.nlm.nih.gov/pubmed/6127716 PMID: 6127716
  
  
• Williams DS: Rhabdom size and photoreceptor membrane turnover in a muscoid fly. Cell and Tissue Research 226:629-639, 1982.
  http://www.ncbi.nlm.nih.gov/pubmed/7139695 PMID: 7139695
  
  
• Williams DS: Photoreceptor membrane shedding and assembly can be initiated locally within an insect eye. Science 218:898-900 (plus front cover), 1982.
  http://www.ncbi.nlm.nih.gov/pubmed/7134980 PMID: 7134980
  
  
• Williams DS: Ommatidial structure in relation to photoreceptor membrane turnover in a locust. Cell and Tissue Research 225:595-617, 1982.
  http://www.ncbi.nlm.nih.gov/pubmed/7127410 PMID: 7127410
  
  
• Blest AD, Hardie RC, McIntyre P, Williams DS: The spectral sensitivities of identified receptors and the function of retinal tiering in the principal eyes of a jumping spider. Journal of Comparative Physiology A 145:227-239, 1981.
  http://link.springer.com/content/pdf/10.1007/BF00605035.pdf
  
  
• Williams DS: Twisted rhabdomeres in the compound eye of the tipulid fly (Diptera). Cell and Tissue Research 217:625-632, 1981.
  http://www.ncbi.nlm.nih.gov/pubmed/7249053 PMID: 7249053
  
  
• Williams DS: A jumping spider's view. Hemisphere 26:178-183, 1981.
  
  
• Blest AD, Williams DS, Kao L: The posterior median eyes of the dinopid spider Menneus. Cell and Tissue Research 211:391-403, 1980.
  http://www.ncbi.nlm.nih.gov/pubmed/7417991 PMID: 7417991
  
  
• Williams DS, McIntyre P: The principal eyes of a jumping spider have a telephoto component. Nature 288:578-580, 1980.
  http://www.www.nature.com/nature/journal/v288/n5791/abs/288578a0.html PMID: 
  
  
• Williams DS, Blest AD: Extracellular shedding of photoreceptor membrane in the open rhabdom of a tipulid fly. Cell and Tissue Research 205:423-438, 1980.
  http://www.ncbi.nlm.nih.gov/pubmed/7357581 PMID: 7357581
  
  
• Williams DS: Organisation of the compound eye of a tipulid fly during the day and night. Zoomorphologie 95:85-104, 1980.
  http://link.springer.com/content/pdf/10.1007/BF00997903.pdf
  
  
• Williams DS: Ca2+-induced structural changes in photoreceptor microvilli of Diptera. Cell and Tissue Research 206:225-232, 1980.
  http://www.ncbi.nlm.nih.gov/pubmed/6771011 PMID: 6771011
  
  
• Williams DS: The feeding behaviour of New Zealand Dolomedes species (Aranaea; Pisauridae). New Zealand Journal of Zoology 6:95-105, 1979.
  http://www.tandfonline.com/doi/abs/10.1080/03014223.1979.10428352
  
  
• Williams DS: The physiological optics of a nocturnal semi-aquatic spider, Dolomedes aquaticus (Pisauridae). Zeitschrift fuer Naturforschung 34c:463-469, 1979.
  http://www.degruyter.com/view/j/znc.1979.34.issue-5-6/znc-1979-5-625/znc-1979-5-625.xml
  
  

Books, Book Chapters, and Special Editions

• Lopes VS, Williams DS: Gene therapy for the retinal degeneration of Usher syndrome due to mutations in MYO7A. Retinal Disorders Genetic Approaches to Diagnosis and treatment. Cold Spring Harbor Perspectives in Medicine 5(6), 2015. doi: 10.1101/cshperspect.a017319.
  http://www.ncbi.nlm.nih.gov/pubmed/25605753 PMID: 25605753
  
  
• Sernagor E, Williams DS: Strategies for restoring sight in retinal dystrophies. Guest Editors. Visual Neuroscience 31:287-288, 2014.
  http://www.ncbi.nlm.nih.gov/pubmed/25346971 PMID: 25346971
  
  
• Lopes VS, Diemer T, Williams DS: Assessment of different virus-mediated approaches for retinal gene therapy of Usher 1B. Advances in Experimental Medicine and Biology 801:725-31, 2014.
  http://www.ncbi.nlm.nih.gov/pubmed/24664764 PMID: 24664764
  
  
• Esteve-Rudd J, Lopes VS, Jiang M, Williams DS: In vivo and in vitro monitoring of phagosome maturation in retinal pigment epithelium cells. Advances in Experimental Medicine and Biology 801:85-90, 2014.
  http://www.ncbi.nlm.nih.gov/pubmed/24664684 PMID: 24664684
  
  
• Williams DS, Lopes VS: Gene Therapy Strategies for Usher Syndrome Type 1B. Advances in Experimental Medicine and Biology 723:235-42, 2012.
  http://www.ncbi.nlm.nih.gov/pubmed/22183338 PMID: 22183338
  
  
• Trivedi D, Williams DS: Ciliary transport of opsin. Advances in Experimental Medicine and Biology 664:185-191, 2010.
  http://www.ncbi.nlm.nih.gov/pubmed/20238016 PMID: 20238016
  
  
• Williams DS, Anderson DH: Rod and cone photoreceptor cells: outer segment membrane renewal. In: Encyclopedia of the Eye, Vol 4. Ed: Dartt, DA. Academic Press, Oxford, UK, pp. 143-146, 2010.
  http://www.sciencedirect.com/science/article/pii/B9780123742032001755 
  
  
• Anderson DH, Williams DS: Rod and cone photoreceptor cells: inner and outer segments. In: Encyclopedia of the Eye, Vol 4. Ed: Dartt, DA. Academic Press, Oxford, UK, pp. 139-142, 2010.
  http://www.sciencedirect.com/science/article/pii/B9780123742032001731 
  
  
• Diemer T, Gibbs D, Williams DS: Analysis of the rate of disk membrane digestion by cultured RPE cells. Advances in Experimental Medicine and Biology 613:321-326, 2008.
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• Williams DS: Retinal degeneration in Usher syndrome. In: Retinal Degenerations: Biology, Diagnostics, and Therapeutics. Eds: Barnstable C, Tombran-Tink J. Humana Press, New Jersey, pp. 137-148, 2007.
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• Lillo C, Kitamoto J, Williams DS: Roles and interactions of Usher 1 proteins in the outer retina. Advances in Experimental Medicine and Biology 572:341-348, 2006.
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• Williams DS, editor: Photoreceptor Cell Biology and Inherited Retinal Degenerations. World Scientific Publishing, Singapore, 2004.
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• Williams DS, Gibbs D: Myosin VIIa in the retina. In: Photoreceptor Cell Biology and Inherited Retinal Degenerations. Ed: Williams DS. World Scientific Publishing, Singapore, pp. 397-436, 2004.
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• Williams DS: Cell biology of photoreceptors and the RPE: A brief introduction. In: Photoreceptor Cell Biology and Inherited Retinal Degenerations. Ed: Williams DS. World Scientific Publishing, Singapore, pp. 1-6, 2004.
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• Gibbs D, Williams DS: Isolation and culture of primary mouse retinal pigmented epithelial cells. Advances in Experimental Medicine and Biology 533:347-352, 2003.
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• Slatter T, Azarian SM, Tebbutt S, Maw M, Williams DS: Screen for Usher syndrome 1B mutations in the ovine myosin VIIa gene. Advances in Experimental Medicine and Biology 533:151-155, 2003.
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• Lillo C, Kitamoto J, Liu X, Quint E, Steel KP, Williams DS: Mouse models for Usher syndrome 1B. Advances in Experimental Medicine and Biology 533:143-150, 2003.
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• Williams DS, Liu X, Vansant G, Ondek, B: Blindness in Usher syndrome 1B: myosin VIIa in the retina. In: Retinal Degenerative Diseases and Experimental Therapy. Eds: Hollyfield JG, Anderson RE, and LaVail MM, Plenum Press, New York, pp 15-26, 1999.
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