Posts classified under: Visual Neurosciences

David Williams, Ph.D.

Publications

A selected list of publications:

Esteve-Rudd Julian, Hazim Roni A, Diemer Tanja, Paniagua Antonio E, Volland Stefanie, Umapathy Ankita, Williams David S   Defective phagosome motility and degradation in cell nonautonomous RPE pathogenesis of a dominant macular degeneration Proceedings of the National Academy of Sciences of the United States of America, 2018; 115(21): 5468-5473.
Hazim Roni A, Williams David S   Cell Culture Analysis of the Phagocytosis of Photoreceptor Outer Segments by Primary Mouse RPE Cells Methods in molecular biology (Clifton, N.J.), 2018; 1753(3): 63-71.
Volland Stefanie, Williams David S   Preservation of Photoreceptor Nanostructure for Electron Tomography Using Transcardiac Perfusion Followed by High-Pressure Freezing and Freeze-Substitution Advances in experimental medicine and biology, 2018; 1074(3): 603-607.
Hazim Roni A, Karumbayaram Saravanan, Jiang Mei, Dimashkie Anupama, Lopes Vanda S, Li Douran, Burgess Barry L, Vijayaraj Preethi, Alva-Ornelas Jackelyn A, Zack Jerome A, Kohn Donald B, Gomperts Brigitte N, Pyle April D, Lowry William E, Williams David S   Differentiation of RPE cells from integration-free iPS cells and their cell biological characterization Stem cell research & therapy, 2017; 8(1): 217.
Galloway Chad A, Dalvi Sonal, Hung Sandy S C, MacDonald Leslie A, Latchney Lisa R, Wong Raymond C B, Guymer Robyn H, Mackey David A, Williams David S, Chung Mina M, Gamm David M, Pébay Alice, Hewitt Alex W, Singh Ruchira   Drusen in patient-derived hiPSC-RPE models of macular dystrophies Proceedings of the National Academy of Sciences of the United States of America, 2017; 114(39): E8214-E8223.
Goldberg Andrew F X, Moritz Orson L, Williams David S   Molecular basis for photoreceptor outer segment architecture Progress in retinal and eye research, 2016; 55(1): 52-81.
Hazim Roni, Jiang Mei, Esteve-Rudd Julian, Diemer Tanja, Lopes Vanda S, Williams David S   Live-Cell Imaging of Phagosome Motility in Primary Mouse RPE Cells Advances in experimental medicine and biology, 2016; 854(30): 751-5.
Volland Stefanie, Hughes Louise C, Kong Christina, Burgess Barry L, Linberg Kenneth A, Luna Gabriel, Zhou Z Hong, Fisher Steven K, Williams David S   Three-dimensional organization of nascent rod outer segment disk membranes Proceedings of the National Academy of Sciences of the United States of America, 2015; 112(48): 14870-5.
Jiang Mei, Esteve-Rudd Julian, Lopes Vanda S, Diemer Tanja, Lillo Concepción, Rump Agrani, Williams David S   Microtubule motors transport phagosomes in the RPE, and lack of KLC1 leads to AMD-like pathogenesis The Journal of cell biology, 2015; 210(4): 595-611.
Volland Stefanie, Esteve-Rudd Julian, Hoo Juyea, Yee Claudine, Williams David S   A comparison of some organizational characteristics of the mouse central retina and the human macula PloS one, 2015; 10(4): e0125631.
Eblimit Aiden, Nguyen Thanh-Minh T, Chen Yiyun, Esteve-Rudd Julian, Zhong Hua, Letteboer Stef, Van Reeuwijk Jeroen, Simons David L, Ding Qian, Wu Ka Man, Li Yumei, Van Beersum Sylvia, Moayedi Yalda, Xu Huidan, Pickard Patrick, Wang Keqing, Gan Lin, Wu Samuel M, Williams David S, Mardon Graeme, Roepman Ronald, Chen Rui   Spata7 is a retinal ciliopathy gene critical for correct RPGRIP1 localization and protein trafficking in the retina Human molecular genetics, 2015; 24(6): 1584-601.
Lopes Vanda S, Williams David S   Gene Therapy for the Retinal Degeneration of Usher Syndrome Caused by Mutations in MYO7A Cold Spring Harbor perspectives in medicine, 2015; 5(6): 1584-601.
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.

Joshua Trachtenberg, Ph.D.

Biography

Joshua Trachtenberg’s research seeks to understand how sensory experiences are written into the fabric of our brains.  Genetics plays a dominant role in wiring together the connections between neurons that establish neural circuitry.  After we are born, our brains are bombarded with information from the world around us.  This sensory information changes neural circuitry, allowing us to learn a language, perform complex visual discrimination, obtain sophisticated motor skills, and learn the subtleties of social interactions.  How this external sensory information instructs neural circuitry is not known.  Given its centrality to complex thought, tackling this question is of some significance.  The Trachtenberg lab employs a sophisticated array of vital imaging and physiological tools to reveal the richness and mechanisms of this experience-dependent plasticity.

Xian-Jie Yang, Ph.D.

Biography

Xian-Jie Yang is a developmental biologist, who has served on the UCLA School of Medicine faculty since she joined the Department of Ophthalmology in 1996. She is a member of the Molecular Biology Institute, Brain Research Institute, and has become an associate professor in 2003. Dr, Yang obtained her B.S in biology from Beijing Normal University and her Ph.D. from Cornell University in Biochemistry, Cell and Molecular Biology after studying transcription regulation. She pursued her postdoctoral training at Harvard University and Harvard Medical School working on vertebrate neural development. Her research centers on development and diseases of the vertebrate retina, in particular growth factor signaling mechanisms during normal neural differentiation and neuronal survival in retinal degeneration. Her laboratory is also developing cellular and molecular therapies for inherited retinal diseases.