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.
https://www.ncbi.nlm.nih.gov/pubmed/35868562 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: 10.1523/JNEUROSCI.0357-21.2021
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: 34262913 PMCID: PMC8273544 DOI: 10.3389/fcell.2021.701853
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: 33547385 PMCID: PMC7864947 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.
PMID: 33106502 PMCID: PMC7588439 DOI: 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, 3(7):e201900620, 2020.
PMID: 32499316 PMCID: PMC7283135 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 (15): jcs242214, 2020.
PMID: 32661088 PMCID: PMC7420818 DOI: 10.1242/jcs.242214
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: 32105772 PMCID: DOI: 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, 117:4400-4410, 2020.
PMID: 32041874 PMCID: PMC7049111 DOI: 10.1073/pnas.1912513117
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: 31577510 PMCID: PMC6962520 DOI: 10.1152/ajpcell.00185.2019
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: 30975916 PMCID: PMC6550008 DOI: 10.1242/jcs.229526
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: 30336127 PMCID: PMC6360117 DOI: 10.1016/j.exer.2018.10.009
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: 10.1007/978-3-319-75402-4_73
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: 29735674 PMCID: PMC6003516 DOI: 10.1073/pnas.1709211115
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: 28969679 PMCID: PMC5625837 DOI: 10.1186/s13287-017-0652-9
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: 28878022 PMCID: PMC5625924 DOI: 10.1073/pnas.1710430114
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: 26960254 PMCID: PMC4792956 DOI: 10.1038/ncomms10972
Goldberg AG, Moritz OL, Williams DS: Molecular basis for photoreceptor outer segment architecture. Progress in Retinal and Eye Research, 55:52-81, 2016.
PMID: 27260426 PMCID: PMC5112118 DOI: 10.1016/j.preteyeres.2016.05.003
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: 28054582 PMCID: PMC6055994 DOI: 10.1038/gt.2016.81
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: 26578801 PMCID: PMC4672767 DOI: 10.1073/pnas.1516309112
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: 26261180 PMCID: PMC4539993 DOI: 10.1083/jcb.201410112
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: 25923208 PMCID: PMC4414478 DOI: 10.1371/journal.pone.0125631
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: 25398945 PMCID: PMC4351378 DOI: 0.1093/hmg/ddu573
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: 25301234 PMCID: PMC5531606 DOI: 10.1007/s12035-014-8920-5
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: 24814193 PMCID: PMC4121470 DOI: 10.1016/j.ajhg.2014.04.010
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: 24619649 PMCID: PMC4173073 DOI: 10.1002/cm.21173
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: 24573290 PMCID: PMC3935090 DOI: 10.1523/JNEUROSCI.1503-13.2014
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: 24047104 PMCID: PMC4005490 DOI: 10.1089/ten.tec.2013.0246
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: 24270420 PMCID: PMC3859425 DOI: 10.1172/JCI69000
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: 23891469 PMCID: PMC3738827 DOI: 10.1016/j.ajhg.2013.06.007
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: 23611523 PMCID: PMC3912860 DOI: 10.1017/S0952523813000096
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: 23344065 PMCID: PMC3640772 DOI: 10.1038/gt.2013.3
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: 22451473 PMCID: PMC3598361 DOI: 10.1093/gerona/gls070
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: 22855808 PMCID: PMC3428073 DOI: 10.1523/JNEUROSCI.0015-12.2012
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: 22560226 PMCID: PMC3565468 DOI: 10.1016/j.cmet.2012.03.019
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: 22391151 PMCID: PMC3694443 DOI: 10.1017/S0952523812000041
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: 22199362 PMCID: PMC3322886 DOI: 10.1074/jbc.M111.256073
Williams DS, Lopes VS: The many different cellular functions of MYO7A in the retina. Biochemical Society transactions, 39:1207-10, 2011.
PMID: 21936790 PMCID: PMC3703834 DOI: 10.1042/BST0391207
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: 21873662 PMCID: PMC3263772 DOI: 10.1167/iovs.11-8313
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: 21757509 PMCID: PMC3209648 DOI: 10.1242/dmm.006429
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: 21493626 PMCID: PMC3110002 DOI: 10.1093/hmg/ddr155
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: 20926680 PMCID: PMC2993738 DOI: 10.1091/mbc.E10-08-0715
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: 20835237 PMCID: PMC2947620 DOI: 10.1038/ng.662
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: 20709808 PMCID: PMC3115666 DOI: 10.1093/hmg/ddq341
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: 20081859 PMCID: PMC2884967 DOI: 10.1038/ng.519
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: 19643958 PMCID: PMC2868451 DOI: 10.1167/iovs.09-4032
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: 20016096 PMCID: PMC2834289 DOI: 10.1523/JNEUROSCI.4876-09.2009
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: 19801628 PMCID: PMC2787325 DOI: 10.1074/jbc.M109.035022
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: 19906976 PMCID: PMC2788486 DOI: 10.1523/JNEUROSCI.3976-09.2009
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: 19793063 PMCID: PMC2988682 DOI: 10.1111/j.1474-9726.2009.00527.x
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: 19447093 PMCID: PMC2691393 DOI: 10.1016/j.neuron.2009.04.006
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: 19420244 PMCID: PMC3849468 DOI: 10.1523/JNEUROSCI.0780-09.2009
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: 19324852 PMCID: PMC2884175 DOI: 10.1167/iovs.09-3471
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: 19324851 PMCID: PMC2893298 DOI: 10.1167/iovs.08-3358
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: 19240024 PMCID: PMC2667774 DOI: 10.1074/jbc.M808857200
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: 18694898 PMCID: PMC2722897 DOI: 10.1093/hmg/ddn240
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: 18654668 PMCID: PMC2483685 DOI: 10.1172/JCI35891
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: 18463160 PMCID: PMC2733815 DOI: 10.1093/hmg/ddn140
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: 18414684 PMCID: PMC2298836 DOI: 10.1172/JCI34316
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: 18326740 PMCID: PMC4505835 DOI: 10.1167/iovs.07-1234
Williams DS: Usher syndrome: animal models, retinal function of Usher proteins, and prospects for gene therapy. Vision Research, 48:433-441, 2008.
PMID: 17936325 PMCID: PMC2680226 DOI: 10.1016/j.visres.2007.08.015
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: 17352418 PMCID: DOI: 10.1002/cm.20198
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: 17268537 PMCID: DOI: 10.1038/sj.gt.3302897
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: 17260955 PMCID: PMC4472003 DOI: 10.1021/bi061466i
Schubert D, Williams DS: ‘Cisgenic’ as a product designation. Nature Biotechnology, 24:1327-1329, 2006.
PMID: 17093469 PMCID: DOI: 10.1038/nbt1106-1327
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: 17065525 PMCID: PMC1904505 DOI: 10.1167/iovs.06-0032
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: 16632484 PMCID: PMC1592550 DOI: 10.1093/hmg/ddl107
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: 16682973 PMCID: DOI: 10.1038/ng1786
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: 16481439 PMCID: PMC2712835 DOI: 10.1523/JNEUROSCI.4251-05.2006
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: 16512666 PMCID: DOI: 10.1021/pr0502323
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: 16337628 PMCID: DOI: 10.1016/j.exer.2005.10.026
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: 16219026 PMCID: DOI: 10.1111/j.1471-4159.2005.03460.x
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: 16043481 PMCID: PMC1249479 DOI: 10.1074/jbc.M505827200
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: 15978262 PMCID: DOI: 10.1016/j.exer.2005.02.014
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: 15749821 PMCID: PMC554798 DOI: 10.1073/pnas.0407698102
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: 15731448 PMCID: DOI: 10.1126/science.1105681
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: 15723066 PMCID: DOI: 10.1038/ng1520
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: 15914637 PMCID: PMC4505833 DOI: 10.1167/iovs.04-1427
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: 15572405 PMCID: PMC2942070 DOI: 10.1242/jcs.01580
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: 15316067 PMCID: DOI: 10.1242/jcs.01316
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: 15233913 PMCID: DOI: 10.1016/j.neuron.2004.06.016
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: 15057245 PMCID: DOI: 10.1038/nature02483
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: 14609561 PMCID: DOI: 10.1016/j.exer.2003.07.007
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: 12809555 PMCID: PMC1223618 DOI: 10.1042/BJ20030408
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: 12743369 PMCID: PMC164472 DOI: 10.1073/pnas.1130432100
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: 12682084 PMCID: PMC2172877 DOI: 10.1083/jcb.200301026
Udovichenko IP, Gibbs D, Williams DS: Actin-based motor properties of native myosin VIIa. Journal of Cell Science, 115:445-450, 2002.
PMID: 11839794 PMCID: DOI:
Williams DS: Transport to the photoreceptor outer segment by myosin VIIa and kinesin II. Vision Research, 42:455-462, 2002.
PMID: 11853761 PMCID: DOI: 10.1016/s0042-6989(01)00228-0
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: 11180984 PMCID: DOI: 10.1006/exer.2000.0963
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: 10943838 PMCID: DOI: 10.1016/s0092-8674(00)00023-4
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: 10414956 PMCID: PMC6782817 DOI: 10.1523/JNEUROSCI.19-15-06267.1999
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: 10842588 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: 9678669 PMCID: DOI: 10.1002/(SICI)1097-0169(1998)40:33.0.CO;2-G
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: 9620764 PMCID: DOI: 10.1038/470
Udovichenko IP, Newton AC, Williams DS: Regulation of the phosphorylation state of rhodopsin by dopamine. Journal of Biological Chemistry, 273:7181-7184, 1998.
PMID: 9516406 PMCID: DOI: 10.1074/jbc.273.13.7181
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: 9227854 PMCID: DOI: 10.1002/(SICI)1097-0169(1997)37:33.0.CO;2-A
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: 9326298 PMCID: DOI: 10.1046/j.1471-4159.1997.69041693.x
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: 9184167 PMCID: DOI: 10.1021/bi9627370
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: 9099669 PMCID: DOI: 10.1074/jbc.272.16.10341
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: 9065465 PMCID: DOI: 10.1074/jbc.272.12.7952
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: 9372448 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: 9068368 PMCID: DOI: 10.1006/exer.1996.0155
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: 8956007 PMCID: DOI: 10.1002/(SICI)1097-0169(1996)35:43.0.CO;2-3
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: 8832403 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: 7592650 PMCID: DOI: 10.1074/jbc.270.41.24375
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: 7896814 PMCID: DOI: 10.1074/jbc.270.12.6710
Azarian SM, Williams DS: Calpain activity in the retinas of normal and RCS rats. Current Eye Research, 14:731-735, 1995.
PMID: 8529410 PMCID: DOI: 10.3109/02713689508998502
Williams DS: The dynamics of cytosolic calcium in photoreceptor cells. Bioessays, 17: 282-286, 1995.
PMID: 7741721 PMCID: DOI: 10.1002/bies.950170403
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: 7925714 PMCID: DOI: 10.1006/exer.1994.1072
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: 7693871 PMCID: DOI: 10.1111/j.1471-4159.1993.tb09831.x
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: 8349693 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: 8236436 PMCID: DOI: 10.1016/0968-0004(93)90032-i
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: 8408304 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: 8491566 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: 1730774 PMCID: PMC2289314 DOI: 10.1083/jcb.116.3.683
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: 1569127 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: 1730772 PMCID: PMC2289304 DOI: 10.1083/jcb.116.3.659
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: 1429904 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: 1526748 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: 1901797 PMCID: DOI: 10.1016/0014-4835(91)90089-w
Newton AC, Williams DS: Involvement of protein kinase C in the phosphorylation of rhodopsin. Journal of Biological Chemistry, 266:17725-17728, 1991.
PMID: 1917916 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: 2004431 PMCID: DOI: 10.1002/cm.970180103
Williams DS: Actin filaments and photoreceptor membrane turnover. Bioessays, 13:171-178, 1991.
PMID: 1859395 PMCID: DOI: 10.1002/bies.950130405
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: 2112548 PMCID: PMC2116135 DOI: 10.1083/jcb.110.6.1993
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: 2341633 PMCID: DOI: 10.1002/cne.902950113
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: 2311685 PMCID: DOI: 10.1016/0014-4835(90)90236-n
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: 2808754 PMCID: DOI: 10.1002/cne.902880410
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: 2551708 PMCID: DOI: 10.1016/0014-4835(89)90052-3
Anderson DH, Williams DS, Neitz J, Farris RN, Fliesler SJ: Tunicamycin-induced degeneration in cone photoreceptors. Visual Neuroscience, 1:153-158, 1988.
PMID: 3154792 PMCID: DOI: 10.1017/s0952523800001425
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: 3397406 PMCID: DOI: 10.1002/cne.902720202
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: 3428393 PMCID: DOI: 10.1016/s0014-4835(87)80117-3
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: 2439472 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: 3804649 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: 3516917 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:
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.
PMID: PMCID: DOI:
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.
PMID: 6127716 PMCID: DOI: 10.1098/rspb.1982.0054
Williams DS: Rhabdom size and photoreceptor membrane turnover in a muscoid fly. Cell and Tissue Research, 226:629-639, 1982.
PMID: 7139695 PMCID: DOI: 10.1007/BF00214790
Williams DS: Photoreceptor membrane shedding and assembly can be initiated locally within an insect eye. Science, 218:898-900 (plus front cover), 1982.
PMID: 7134980 PMCID: DOI: 10.1126/science.7134980
Williams DS: Ommatidial structure in relation to photoreceptor membrane turnover in a locust. Cell and Tissue Research, 225:595-617, 1982.
PMID: 7127410 PMCID: DOI: 10.1007/BF00214807
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:
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.
PMID: 7249053 PMCID: DOI: 10.1007/BF00219369
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: 7417991 PMCID: DOI: 10.1007/BF00234395
Williams DS, McIntyre P: The principal eyes of a jumping spider have a telephoto component. Nature, 288:578-580, 1980.
PMID: PMCID: DOI: 10.1038/288578a0
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: 7357581 PMCID: DOI: 10.1007/BF00232283
Williams DS: Organisation of the compound eye of a tipulid fly during the day and night. Zoomorphologie, 95:85-104, 1980.
PMID: PMCID: DOI:
http://link.springer.com/content/pdf/10.1007/BF00997903.pdf
Williams DS: Ca++-induced structural changes in photoreceptor microvilli of Diptera. Cell and Tissue Research, 206:225-232, 1980.
PMID: 6771011 PMCID: DOI: 10.1007/BF00232766
Williams DS: The feeding behaviour of New Zealand Dolomedes species (Aranaea; Pisauridae). New Zealand Journal of Zoology, 6:95-105, 1979.
PMID: PMCID: DOI: 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.
PMID: PMCID: DOI: 10.1515/znc-1979-5-625
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: 31884643 PMCID: DOI: 10.1007/978-3-030-27378-1_64
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: 29721993 PMCID: PMC6501585 DOI: 10.1007/978-3-319-75402-4_73
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: 29564781 PMCID: PMC6531314 DOI: 10.1007/978-1-4939-7720-8_4
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: 26427485 PMCID: DOI: 10.1007/978-3-319-17121-0_100
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: 25605753 PMCID: DOI: 10.1101/cshperspect.a017319
Sernagor E, Williams DS: Treatment strategies for retinal dystrophies. Guest Editors. Visual Neuroscience, 31:287-288, 2014.
PMID: 25346971 PMCID: DOI: 10.1017/s0952523814000273
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: 24664764 PMCID: DOI: 10.1007/978-1-4614-3209-8_91
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: 24664684 PMCID: DOI: 10.1007/978-1-4614-3209-8_11
Williams DS, Lopes VS: Gene Therapy Strategies for Usher Syndrome Type 1B. Advances in Experimental Medicine and Biology, 723:235-42, 2012.
PMID: 22183338 PMCID: DOI: 10.1007/978-1-4614-0631-0_31
Trivedi D, Williams DS: Ciliary transport of opsin. Advances in Experimental Medicine and Biology, 664:185-191, 2010.
PMID: 20238016 PMCID: PMC2933454 DOI: 10.1007/978-1-4419-1399-9_21
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: 10.1016/B978-0-12-374203-2.00175-5
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: 10.1016/B978-0-12-374203-2.00173-1
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: 10.1007/978-0-387-74904-4_37
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: 10.1007/978-1-59745-186-4_7
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: 10.1007/0-387-32442-9_48
Williams DS, editor: Photoreceptor Cell Biology and Inherited Retinal Degenerations. World Scientific Publishing, Singapore, 2004.
PMID: PMCID: DOI: doi.org/10.1142/5557
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: 10.1142/9789812561756_0015
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: 10.1142/9789812561756_0001
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: 10.1007/978-1-4615-0067-4_44
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: 10.1007/978-1-4615-0067-4_19
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: 10.1007/978-1-4615-0067-4_18
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: 10.1007/978-0-585-33172-0_2