All Publications

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

Links to other publications
Selected Publications, 1980 - Present
Publications Most Related to Current Research

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