Selected Publications
Selected Publications, 1980-Present
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Publications Most Related to Current Research
Umapathy A, Torten G, Paniagua, AE, Chung J, Tomlinson M, Lim C, Williams DS: Spatiotemporal live-cell analysis of photoreceptor outer segment membrane ingestion by the retinal pigment epithelium reveals actin-regulated scission. Journal of Neuroscience 43:2653-2664, 2023.
https://www.ncbi.nlm.nih.gov/pubmed/36878726 doi: 10.1523/JNEUROSCI.1726-22.2023
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
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
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
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
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
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
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
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
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
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
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
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
Schubert D, Williams DS: ‘Cisgenic’ as a product designation.Nature Biotechnology, 24:1327-1329, 2006.
PMID: 17093469 PMCID: DOI: 10.1038/nbt1106-1327
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
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
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
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
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
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
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
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
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: 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, McIntyre P: The principal eyes of a jumping spider have a telephoto component. Nature, 288:578-580, 1980.
PMID: PMCID: DOI: 10.1038/288578a0