David S. Williams, Ph.D.

Frost Laura S, Lopes Vanda S, Bragin Alvina, Reyes-Reveles Juan, Brancato Jennifer, Cohen Art, Mitchell Claire H, Williams David S, Boesze-Battaglia Kathleen   The Contribution of Melanoregulin to Microtubule-Associated Protein 1 Light Chain 3 (LC3) Associated Phagocytosis in Retinal Pigment Epithelium Molecular neurobiology, 2014; .

Crouse Jacquelin A, Lopes Vanda S, Sanagustin Jovenal T, Keady Brian T, Williams David S, Pazour Gregory J   Distinct functions for IFT140 and IFT20 in opsin transport Cytoskeleton (Hoboken, N.J.), 2014; 71(5): 302-10.

Almenar-Queralt Angels, Falzone Tomas L, Shen Zhouxin, Lillo Concepcion, Killian Rhiannon L, Arreola Angela S, Niederst Emily D, Ng Kheng S, Kim Sonia N, Briggs Steven P, Williams David S, Goldstein Lawrence S B   UV irradiation accelerates amyloid precursor protein (APP) processing and disrupts APP axonal transport The Journal of neuroscience : the official journal of the Society for Neuroscience, 2014; 34(9): 3320-39.

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, 2013; .

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 , 2012; 287: 11469-11480.

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, 2012; 29: 83-93.

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, 2012; 32: 10587-10593.

Da Cruz Sandrine, Parone Philippe A, Lopes Vanda S, Lillo Concepción, McAlonis-Downes Melissa, Lee Sandra K, Vetto Anne P, Petrosyan Susanna, Marsala Martin, Murphy Anne N, Williams David S, Spiegelman Bruce M, Cleveland Don W   Elevated PGC-1α activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS Cell metabolism, 2012; 15(5): 778-86.

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 , 2011; 4: 786-800.

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, 2011; 52: 7924-7936.

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, 2011; 20: 2560-2570.

Williams DS, Lopes VS   The many different cellular functions of MYO7A in the retina Biochemical Society transactions, 2011; 39: 1207-10.

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 Al, 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 , 2010; 42: 175-180.

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, 2010; 21: 4076-4088.

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, 2010; 51: 1130-1135.

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 , 2010; 19: 4229-4238.

Louie Carrie M, Caridi Gianluca, Lopes Vanda S, Brancati Francesco, Kispert Andreas, Lancaster Madeline A, Schlossman Andrew M, Otto Edgar A, Leitges Michael, Gröne Hermann-Josef, Lopez Irma, Gudiseva Harini V, O'Toole John F, Vallespin Elena, Ayyagari Radha, Ayuso Carmen, Cremers Frans P M, den Hollander Anneke I, Koenekoop Robert K, Dallapiccola Bruno, Ghiggeri Gian Marco, Hildebrandt Friedhelm, Valente Enza Maria, Williams David S, Gleeson Joseph G   AHI1 is required for photoreceptor outer segment development and is a modifier for retinal degeneration in nephronophthisis Nature genetics, 2010; 42(2): 175-80.

Williams David S, Cash Alan, Hamadani Lara, Diemer Tanja   Oxaloacetate supplementation increases lifespan in Caenorhabditis elegans through an AMPK/FOXO-dependent pathway Aging cell, 2009; 8(6): 765-8.

Gibbs Daniel, Cideciyan Artur V, Jacobson Samuel G, Williams David S   Retinal pigment epithelium defects in humans and mice with mutations in MYO7A: imaging melanosome-specific autofluorescence Investigative ophthalmology & visual science, 2009; 50(9): 4386-93.

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, 2009; 29: 15810-15818.

Falzone Tomás L, Stokin Gorazd B, Lillo Concepción, Rodrigues Elizabeth M, Westerman Eileen L, Williams David S, Goldstein Lawrence S B   Axonal stress kinase activation and tau misbehavior induced by kinesin-1 transport defects The Journal of neuroscience : the official journal of the Society for Neuroscience, 2009; 29(18): 5758-67.

Damek-Poprawa Monika, Diemer Tanja, Lopes Vanda S, Lillo Concepción, Harper Dawn C, Marks Michael S, Wu Yalin, Sparrow Janet R, Rachel Rivka A, Williams David S, Boesze-Battaglia Kathleen   Melanoregulin (MREG) modulates lysosome function in pigment epithelial cells The Journal of biological chemistry, 2009; 284(16): 10877-89.