Selected Publications, 1980-Present

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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

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