All Publications
All Publications
(140+ journal articles plus book chapters, etc.)
Links to other publications
Selected Publications, 1980 - Present
Publications Most Related to Current Research
Hazim RA, Paniagua AE, Tang L, Yang K, Kim KKO, Stiles L, Divakaruni AS, Williams DS: Vitamin B3, nicotinamide, enhances mitochondrial metabolism to promote differentiation of the retinal pigment epithelium. Journal of Biological Chemistry 298:102286, 2022.
PMID: 35868562. doi: 10.1016/j.jbc.2022.102286
Chadha A, Paniagua AE, Williams DS: Comparison of ciliary targeting of two rhodopsin-like GPCRs: role of C-terminal localization sequences in relation to cilium type. Journal of Neuroscience, in press, 2021.
PMID: PMCID: DOI:
Paniagua AE, Segurado A, Dolon JF, Esteve-Rudd J, Velasco A, Williams DS, Lillo C: Key role for CRB2 in the maintenance of apicobasal polarity in retinal pigment epithelial cells. Frontiers in Cell and Developmental Biology, 9:701853, 2021.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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, 3(7):e201900620, 2020.
PMID: PMCID: DOI:
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 (15): jcs242214, 2020.
PMID: PMCID: DOI:
Lakkaraju A, Umapathy A, Tan LX, Daniele L, Philp NJ, Boesze-Battaglia K, Williams DS: The cell biology of the retinal pigment epithelium.
Progress in Retina and Eye Research, 78:100846, 2020.
PMID: PMCID: DOI:
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, 117:4400-4410, 2020.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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):jcs229526, 2019.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Volland S, Williams DS: 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, 2018.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Goldberg AG, Moritz OL, Williams DS: Molecular basis for photoreceptor outer segment architecture. Progress in Retinal and Eye Research, 55:52-81, 2016.
PMID: PMCID: DOI:
Williams DS, Chadha A, Hazim R, Gibbs D: Gene Therapy approaches for prevention of retinal degeneration in Usher syndrome. Gene Therapy, 24:68-71, 2018.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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, 52(3):1135-1151, 2014.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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: ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption. Journal of Clinical Investigation, 123(12):5179-89, 2013.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Williams DS, Lopes VS: The many different cellular functions of MYO7A in the retina. Biochemical Society transactions, 39:1207-10, 2011.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Williams DS: Usher syndrome: animal models, retinal function of Usher proteins, and prospects for gene therapy. Vision Research, 48:433-441, 2008.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Schubert D, Williams DS: ‘Cisgenic’ as a product designation. Nature Biotechnology, 24:1327-1329, 2006.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Udovichenko IP, Gibbs D, Williams DS: Actin-based motor properties of native myosin VIIa. Journal of Cell Science, 115:445-450, 2002.
PMID: PMCID: DOI:
Williams DS: Transport to the photoreceptor outer segment by myosin VIIa and kinesin II. Vision Research, 42:455-462, 2002.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Udovichenko IP, Newton AC, Williams DS: Regulation of the phosphorylation state of rhodopsin by dopamine. Journal of Biological Chemistry, 273:7181-7184, 1998.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Greene NM, Williams DS, Newton AC: Identification of protein kinase C phosphorylation sites on bovine rhodopsin. Journal of Biological Chemistry, 272:10341-10344, 1997.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Schlamp CS, Williams DS: Myosin V in the retina: localization in the rod photoreceptor synapse. Experimental Eye Research, 63:613-619, 1996.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Azarian SM, Williams DS: Calpain activity in the retinas of normal and RCS rats. Current Eye Research, 14:731-735, 1995.
PMID: PMCID: DOI:
Williams DS: The dynamics of cytosolic calcium in photoreceptor cells. Bioessays, 17: 282-286, 1995.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Nickells RW, Newton AC, Williams DS: PCR reamplification of small target cDNAs from high-resolution polyacrylamide gels. Amplifications, 9:6-7, 1993.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Newton AC, Williams DS: Does protein kinase C play a role in rhodopsin desensitization? Trends in Biochemical Sciences, 18:275-277, 1993.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Arikawa K, Williams DS: Acetylated alpha-tubulin in the connecting cilium of developing rat photoreceptors. Investigative Ophthalmology and Visual Science, 34:2145-2149, 1993.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Williams DS, Hallett M, Arikawa K: Association of myosin with the connecting cilium of rod photoreceptors. Journal of Cell Science, 103:183-190, 1992.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Newton AC, Williams DS: Involvement of protein kinase C in the phosphorylation of rhodopsin. Journal of Biological Chemistry, 266:17725-17728, 1991.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Williams DS: Actin filaments and photoreceptor membrane turnover. Bioessays, 13:171-178, 1991.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Williams DS, Linberg KA, Fisher SK: Shedding of cone photoreceptor disk membranes in lizard eyecups in vitro. Experimental Eye Research, 50:231-234, 1990.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Anderson DH, Williams DS, Neitz J, Farris RN, Fliesler SJ: Tunicamycin-induced degeneration in cone photoreceptors. Visual Neuroscience, 1:153-158, 1988.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Shuster TS, Walter AE, Williams DS, Farber DB: Identification of a PNA-binding protein from human retina. Experimental Eye Research, 45:685-694, 1987.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Williams DS: Rhabdom size and photoreceptor membrane turnover in a muscoid fly. Cell and Tissue Research, 226:629-639, 1982.
PMID: PMCID: DOI:
Williams DS: Photoreceptor membrane shedding and assembly can be initiated locally within an insect eye. Science, 218:898-900 (plus front cover), 1982.
PMID: PMCID: DOI:
Williams DS: Ommatidial structure in relation to photoreceptor membrane turnover in a locust. Cell and Tissue Research, 225:595-617, 1982.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Williams DS: Twisted rhabdomeres in the compound eye of the tipulid fly (Diptera). Cell and Tissue Research, 217:625-632, 1981.
PMID: PMCID: DOI:
Williams DS: A jumping spider's view. Hemisphere, 26:178-183, 1981.
PMID: PMCID: DOI:
Blest AD, Williams DS, Kao L: The posterior median eyes of the dinopid spider Menneus. Cell and Tissue Research, 211:391-403, 1980.
PMID: PMCID: DOI:
Williams DS, McIntyre P: The principal eyes of a jumping spider have a telephoto component. Nature, 288:578-580, 1980.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Williams DS: Organisation of the compound eye of a tipulid fly during the day and night. Zoomorphologie, 95:85-104, 1980.
PMID: PMCID: DOI:
Williams DS: Ca++-induced structural changes in photoreceptor microvilli of Diptera. Cell and Tissue Research, 206:225-232, 1980.
PMID: PMCID: DOI:
Williams DS: The feeding behaviour of New Zealand Dolomedes species (Aranaea; Pisauridae). New Zealand Journal of Zoology, 6:95-105, 1979.
PMID: PMCID: DOI:
Williams DS: The physiological optics of a nocturnal semi-aquatic spider, Dolomedes aquaticus (Pisauridae). Zeitschrift fuer Naturforschung, 34c:463-469, 1979.
PMID: PMCID: DOI:
Books, Book Chapters, and Special Editions
Umapathy A, Williams DS: Live Imaging of Organelle Motility in RPE Flatmounts. Advances in Experimental Medicine and Biology, 1185:389-393, 2019.
PMID: PMCID: DOI:
Volland S, Williams DS: Preservation of photoreceptor nanostructure for electron tomography using transcardiac perfusion followed by high-pressure freezing and freeze-substitution. Advances in Experimental Medicine and Biology, 1074:603-607, 2018.
PMID: PMCID: DOI:
Hazim RA, Williams DS: Cell culture analysis of the phagocytosis of photoreceptor outer segments by primary mouse RPE cells. In: Tanimoto N. (eds) Mouse Retinal Phenotyping. Methods in Molecular Biology, 1753:63-71. Humana Press, New York, NY, 2018.
PMID: PMCID: DOI:
Hazim RA, Jiang M, Esteve-Rudd J, Diemer T, Lopes VS, Williams DS: Live-cell imaging of phagosome motility in primary mouse RPE cells. Advances in Experimental Medicine and Biology, 854:751-755, 2016.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Sernagor E, Williams DS: Treatment strategies for retinal dystrophies. Guest Editors. Visual Neuroscience, 31:287-288, 2014.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Williams DS, Lopes VS: Gene Therapy Strategies for Usher Syndrome Type 1B. Advances in Experimental Medicine and Biology, 723:235-42, 2012.
PMID: PMCID: DOI:
Trivedi D, Williams DS: Ciliary transport of opsin. Advances in Experimental Medicine and Biology, 664:185-191, 2010.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Williams DS, editor: Photoreceptor Cell Biology and Inherited Retinal Degenerations. World Scientific Publishing, Singapore, 2004.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
Gibbs D, Williams DS: Isolation and culture of primary mouse retinal pigmented epithelial cells. Advances in Experimental Medicine and Biology, 533:347-352, 2003.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI:
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.
PMID: PMCID: DOI: