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David Williams

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Deepti Trivedi; Emilie Colin; Carrie M Louie; David S Williams
Live-cell imaging evidence for the ciliary transport of rod photoreceptor opsin by heterotrimeric kinesin-2.
Sandrine Da Cruz; Sandra K Lee; Concepcion Lillo; Vanda S Lopes; Martin Marsala; Melissa McAlonis-Downes; Anne N Murphy; Philippe A Parone; Susanna Petrosyan; Bruce M Spiegelman; Anne P Vetto; David S Williams; Don W Cleveland
Elevated PGC-1α activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS.
Maren Engelhardt; Vanda S Lopes; Lisa Nguyen; Steven Nusinowitz; Chinatsu Tosha; Bryan Chen; David S Williams
Functional and morphological analysis of the subretinal injection of retinal pigment epithelium cells.

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All Publications

2012: Deepti Trivedi; Emilie Colin; Carrie M Louie; David S Williams
Live-cell imaging evidence for the ciliary transport of rod photoreceptor opsin by heterotrimeric kinesin-2.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2012;32(31):10587-93.
2012: Sandrine Da Cruz; Sandra K Lee; Concepcion Lillo; Vanda S Lopes; Martin Marsala; Melissa McAlonis-Downes; Anne N Murphy; Philippe A Parone; Susanna Petrosyan; Bruce M Spiegelman; Anne P Vetto; David S Williams; Don W Cleveland
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.
2012: Maren Engelhardt; Vanda S Lopes; Lisa Nguyen; Steven Nusinowitz; Chinatsu Tosha; Bryan Chen; David S Williams
Functional and morphological analysis of the subretinal injection of retinal pigment epithelium cells.
Visual neuroscience 2012;29(2):83-93.
2011: Jennifer B Phillips; Srirangan Sampath; Tallafuss Alexandra; Tom A Titus; Philip Washbourne; Bernardo Blanco-Sanchez; Philip F Han; Zachary G Jacobs; Bronya J Keats; Kornnika Khanobdee; Jennifer J Lentz; Monalisa Mishra; David S Williams; Monte Westerfield
Harmonin (Ush1c) is required in zebrafish Müller glial cells for photoreceptor synaptic development and function.
Disease models & mechanisms 2011;4(6):786-800.
2011: David S Williams; Vanda S Lopes
The many different cellular functions of MYO7A in the retina.
Biochemical Society transactions 2011;39(5):1207-10.
2010: Vanda S Lopes; David Jimeno; Kornnika Khanobdee; Xiaodan Song; Bryan Chen; Steven Nusinowitz; David S Williams
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(23):4076-88.
2010: Carrie M Louie; Gianluca Caridi; Vanda S Lopes; Francesco Brancati; Andreas Kispert; Madeline A Lancaster; Andrew M Schlossman; Edgar Otto; Michael Leitges; Hermann-Josef Gröne; Irma Lopez; Harini V Gudiseva; John F O'Toole; Elena Vallespin; Radha Ayyagari; Carmen Ayuso; Frans P M Cremers; Anneke I den Hollander; Robert K Koenekoop; Bruno Dallapiccola; Gian Marco Ghiggeri; Friedhelm Hildebrandt; Enza Maria Valente; David S Williams; Joseph G Gleeson
AHI1 is required for photoreceptor outer segment development and is a modifier for retinal degeneration in nephronophthisis.
Nature genetics 2010;42(2):175-80.
2010: Daniel Gibbs; Tanja Diemer; Kornnika Khanobdee; Jane Hu; Dean Bok; David S Williams
Function of MYO7A in the human RPE and the validity of shaker1 mice as a model for Usher syndrome 1B.
Investigative ophthalmology & visual science 2010;51(2):1130-5.
2010: Deepti Trivedi; David S Williams
Ciliary transport of opsin.
Advances in experimental medicine and biology 2010;664():185-91.
2009: Martin Schwander; Vanda Lopes; Anna Sczaniecka; Daniel Gibbs; Concepcion Lillo; David Delano; Lisa Tarantino; Timothy Wiltshire; David S Williams; Ulrich Müller
A novel allele of myosin VIIa reveals a critical function for the C-terminal FERM domain for melanosome transport in retinal pigment epithelial cells.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2009;29(50):15810-8.
2009: David S Williams; Alan Cash; Lara Hamadani; Tanja Diemer
Oxaloacetate supplementation increases lifespan in Caenorhabditis elegans through an AMPK/FOXO-dependent pathway.
Aging cell 2009;8(6):765-8.
2009: David S Williams; Tomas S Aleman; Concepcion Lillo; Vanda S Lopes; Louise C Hughes; Edwin M Stone; Samuel G Jacobson
Harmonin in the murine retina and the retinal phenotypes of Ush1c-mutant mice and human USH1C.
Investigative ophthalmology & visual science 2009;50(8):3881-9.
2009: Tomás L Falzone; Gorazd B Stokin; Concepcion Lillo; Elizabeth M Rodrigues; Eileen L Westerman; David S Williams; Lawrence S B Goldstein
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.
2008: Gorazd B Stokin; Angels Almenar-Queralt; Shermali Gunawardena; Elizabeth M Rodrigues; Tomás Falzone; Jungsu Kim; Concepcion Lillo; Stephanie L Mount; Elizabeth A Roberts; Eileen McGowan; David S Williams; Lawrence S B Goldstein
Amyloid precursor protein-induced axonopathies are independent of amyloid-beta peptides.
Human molecular genetics 2008;17(22):3474-86.
2008: Kecia L Feathers; Arkady L Lyubarsky; Naheed W Khan; Karen Teofilo; Anand Swaroop; David S Williams; Edward N Pugh; Debra A Thompson
Nrl-knockout mice deficient in Rpe65 fail to synthesize 11-cis retinal and cone outer segments.
Investigative ophthalmology & visual science 2008;49(3):1126-35.
2008: David S Williams
Usher syndrome: animal models, retinal function of Usher proteins, and prospects for gene therapy.
Vision research 2008;48(3):433-41.
2008: Tanja Diemer; Daniel Gibbs; David S Williams
Analysis of the rate of disk membrane digestion by cultured RPE cells.
Advances in experimental medicine and biology 2008;613():321-6.
2007: Adriana E Klomp; Karen Teofilo; Erin Legacki; David S Williams
Analysis of the linkage of MYRIP and MYO7A to melanosomes by RAB27A in retinal pigment epithelial cells.
Cell motility and the cytoskeleton 2007;64(6):474-87.
2007: T Hashimoto; Daniel Gibbs; Concepcion Lillo; Sassan M Azarian; E Legacki; X-M Zhang; Xian-Jie Yang; David S Williams
Lentiviral gene replacement therapy of retinas in a mouse model for Usher syndrome type 1B.
Gene therapy 2007;14(7):584-94.
2006: David Jimeno; Leonard Feiner; Concepcion Lillo; Karen Teofilo; Lawrence S B Goldstein; Eric A Pierce; David S Williams
Analysis of kinesin-2 function in photoreceptor cells using synchronous Cre-loxP knockout of Kif3a with RHO-Cre.
Investigative ophthalmology & visual science 2006;47(11):5039-46.
2006: Bo Chang; Hemant Khanna; Norman L Hawes; David Jimeno; Shirley He; Concepcion Lillo; Sunil K Parapuram; Hong Cheng; Alison Scott; Ronald E Hurd; John A Sayer; Edgar Otto; Massimo Attanasio; John F O'Toole; Genglin Jin; Chengchao Shou; Friedhelm Hildebrandt; David S Williams; John R Heckenlively; Anand Swaroop
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 2006;15(11):1847-57.
2006: Sassan M Azarian; Ian McLeod; Concepcion Lillo; Daniel Gibbs; John R Yates; David S Williams
Proteomic analysis of mature melanosomes from the retinal pigmented epithelium.
Journal of proteome research 2006;5(3):521-9.
2006: David Jimeno; Concepcion Lillo; Elizabeth A Roberts; Lawrence S B Goldstein; David S Williams
Kinesin-2 and photoreceptor cell death: requirement of motor subunits.
Experimental eye research 2006;82(2):351-3.
2006: Concepcion Lillo; Junko Kitamoto; David S Williams
Roles and interactions of usher 1 proteins in the outer retina.
Advances in experimental medicine and biology 2006;572():341-8.
2005: Xuemei Zhu; Kebin Wu; Lawrence Rife; Niamh X Cawley; Bruce Brown; Tiffany Adams; Karen Teofilo; Concepcion Lillo; David S Williams; Y Peng Loh; Cheryl M Craft
Carboxypeptidase E is required for normal synaptic transmission from photoreceptors to the inner retina.
Journal of neurochemistry 2005;95(5):1351-62.
2005: Hemant Khanna; Toby W Hurd; Concepcion Lillo; Xinhua Shu; Sunil K Parapuram; Shirley He; Masayuki Akimoto; Alan F Wright; Benjamin L Margolis; David S Williams; Anand Swaroop
RPGR-ORF15, which is mutated in retinitis pigmentosa, associates with SMC1, SMC3, and microtubule transport proteins.
The Journal of biological chemistry 2005;280(39):33580-7.
2005: Junko Kitamoto; Richard T Libby; Daniel Gibbs; Karen P Steel; David S Williams
Myosin VI is required for normal retinal function.
Experimental eye research 2005;81(1):116-20.
2005: Gorazd B Stokin; Concepcion Lillo; Tomás L Falzone; Richard G Brusch; Edward Rockenstein; Stephanie L Mount; Rema Raman; Peter Davies; Eliezer Masliah; David S Williams; Lawrence S B Goldstein
Axonopathy and transport deficits early in the pathogenesis of Alzheimer's disease.
Science (New York, N.Y.) 2005;307(5713):1282-8.
2004: Daniel Gibbs; Sassan M Azarian; Concepcion Lillo; Junko Kitamoto; Adriana E Klomp; Karen P Steel; Richard T Libby; David S Williams
Role of myosin VIIa and Rab27a in the motility and localization of RPE melanosomes.
Journal of cell science 2004;117(Pt 26):6473-83.
2004: Richard T Libby; Concepcion Lillo; Junko Kitamoto; David S Williams; Karen P Steel
Myosin Va is required for normal photoreceptor synaptic activity.
Journal of cell science 2004;117(Pt 19):4509-15.
2003: Richard T Libby; Junko Kitamoto; Ralph H Holme; David S Williams; Karen P Steel
Cdh23 mutations in the mouse are associated with retinal dysfunction but not retinal degeneration.
Experimental eye research 2003;77(6):731-9.
2003: Daniel Gibbs; Junko Kitamoto; David S Williams
Abnormal phagocytosis by retinal pigmented epithelium that lacks myosin VIIa, the Usher syndrome 1B protein.
Proceedings of the National Academy of Sciences of the United States of America 2003;100(11):6481-6.
2003: Daniel Gibbs; David S Williams
Isolation and culture of primary mouse retinal pigmented epithelial cells.
Advances in experimental medicine and biology 2003;533():347-52.
2003: Tania Slatter; Sassan M Azarian; Scott Tebbutt; Marion A Maw; David S Williams
Screen for usher syndrome 1B mutations in the ovine myosin VIIa gene.
Advances in experimental medicine and biology 2003;533():151-5.
2003: Concepcion Lillo; Junko Kitamoto; Xinran Liu; Elizabeth Quint; Karen P Steel; David S Williams
Mouse models for Usher syndrome 1B.
Advances in experimental medicine and biology 2003;533():143-50.
2002: David S Williams
Transport to the photoreceptor outer segment by myosin VIIa and kinesin II.
Vision research 2002;42(4):455-62.
2002: Igor P Udovichenko; Daniel Gibbs; David S Williams
Actin-based motor properties of native myosin VIIa.
Journal of cell science 2002;115(Pt 2):445-50.
1999: Xinran Liu; Igor P Udovichenko; S D Brown; K P Steel; David S Williams
Myosin VIIa participates in opsin transport through the photoreceptor cilium.
The Journal of neuroscience : the official journal of the Society for Neuroscience 1999;19(15):6267-74.
1998: Igor P Udovichenko; Alexandra C Newton; David S Williams
Regulation of the phosphorylation state of rhodopsin by dopamine.
The Journal of biological chemistry 1998;273(13):7181-4.
1998: U Wolfrum; Xinran Liu; A Schmitt; Igor P Udovichenko; David S Williams
Myosin VIIa as a common component of cilia and microvilli.
Cell motility and the cytoskeleton 1998;40(3):261-71.
1997: Xinran Liu; Tinghuai Wu; S Stowe; A Matsushita; Kentaro Arikawa; M I Naash; David S Williams
Defective phototransductive disk membrane morphogenesis in transgenic mice expressing opsin with a mutated N-terminal domain.
Journal of cell science 1997;110 ( Pt 20)():2589-97.
1997: David S Williams; X Liu; C L Schlamp; B Ondek; S Jaken; A C Newton
Characterization of protein kinase C in photoreceptor outer segments.
Journal of neurochemistry 1997;69(4):1693-702.
1997: N M Greene; David S Williams; Alexandra C Newton
Identification of protein kinase C phosphorylation sites on bovine rhodopsin.
The Journal of biological chemistry 1997;272(16):10341-4.
1997: Igor P Udovichenko; Alexandra C Newton; David S Williams
Contribution of protein kinase C to the phosphorylation of rhodopsin in intact retinas.
The Journal of biological chemistry 1997;272(12):7952-9.
1997: Xinran Liu; G Vansant; Igor P Udovichenko; U Wolfrum; David S Williams
Myosin VIIa, the product of the Usher 1B syndrome gene, is concentrated in the connecting cilia of photoreceptor cells.
Cell motility and the cytoskeleton 1997;37(3):240-52.
1996: Cassandra L Schlamp; David S Williams
Myosin V in the retina: localization in the rod photoreceptor synapse.
Experimental eye research 1996;63(6):613-9.
1996: M A Hallett; J L Delaat; Kentaro Arikawa; Cassandra L Schlamp; F Kong; David S Williams
Distribution of guanylate cyclase within photoreceptor outer segments.
Journal of cell science 1996;109 ( Pt 7)():1803-12.
1996: J L Hicks; X Liu; David S Williams
Role of the ninaC proteins in photoreceptor cell structure: ultrastructure of ninaC deletion mutants and binding to actin filaments.
Cell motility and the cytoskeleton 1996;35(4):367-79.
1995: S M Azarian; A J King; M A Hallett; David S Williams
Selective proteolysis of arrestin by calpain. Molecular characteristics and its effect on rhodopsin dephosphorylation.
The Journal of biological chemistry 1995;270(41):24375-84.
1995: S M Azarian; David S Williams
Calpain activity in the retinas of normal and RCS rats.
Current eye research 1995;14(8):731-5.
1995: David S Williams
The dynamics of cytosolic calcium in photoreceptor cells.
BioEssays : news and reviews in molecular, cellular and developmental biology 1995;17(4):282-6.
1994: David S Williams; S Park; Cassandra L Schlamp; A C Newton
Protein kinase C association with the retinal cytoskeleton and phosphorylation of vimentin.
Experimental eye research 1994;58(6):747-59.
1993: Robert W Nickells; Cassandra L Schlamp; A C Newton; David S Williams
Gene expression of the neuropeptide-processing enzyme carboxypeptidase E in rat photoreceptor cells.
Journal of neurochemistry 1993;61(5):1891-900.
1993: S M Azarian; Cassandra L Schlamp; David S Williams
Characterization of calpain II in the retina and photoreceptor outer segments.
Journal of cell science 1993;105 ( Pt 3)():787-98.
1992: David S Williams; E A Roberts
Modification of the daily photoreceptor membrane shedding response in vitro by antioxidants.
Investigative ophthalmology & visual science 1992;33(10):3005-8.
1992: David S Williams; M A Hallett; Kentaro Arikawa
Association of myosin with the connecting cilium of rod photoreceptors.
Journal of cell science 1992;103 ( Pt 1)():183-90.
1992: Kentaro Arikawa; Laurie L Molday; Robert S Molday; David S Williams
Localization of peripherin/rds in the disk membranes of cone and rod photoreceptors: relationship to disk membrane morphogenesis and retinal degeneration.
The Journal of cell biology 1992;116(3):659-67.
1992: J L Hicks; David S Williams
Distribution of the myosin I-like ninaC proteins in the Drosophila retina and ultrastructural analysis of mutant phenotypes.
Journal of cell science 1992;101 ( Pt 1)():247-54.
1991: David S Williams
Actin filaments and photoreceptor membrane turnover.
BioEssays : news and reviews in molecular, cellular and developmental biology 1991;13(4):171-8.
1991: T Päällysaho; David S Williams
Epithelial cell-substrate adhesion in the cornea: localization of actin, talin, integrin, and fibronection [corrected].
Experimental eye research 1991;52(3):261-7.
1991: Kentaro Arikawa; David S Williams
Alpha-actinin and actin in the outer retina: a double immunoelectron microscopic study.
Cell motility and the cytoskeleton 1991;18(1):15-25.
1990: Kentaro Arikawa; J L Hicks; David S Williams
Identification of actin filaments in the rhabdomeral microvilli of Drosophila photoreceptors.
The Journal of cell biology 1990;110(6):1993-8.
1990: David S Williams; Kentaro Arikawa; T Paallysaho
Cytoskeletal components of the adherens junctions between the photoreceptors and the supportive Müller cells.
The Journal of comparative neurology 1990;295(1):155-64.
1990: David S Williams; Kenneth A Linberg; Steven K Fisher
Shedding of cone photoreceptor disk membranes in lizard eyecups maintained in vitro.
Experimental eye research 1990;50(2):231-3.
1989: Kentaro Arikawa; David S Williams
Organization of actin filaments and immunocolocalization of alpha-actinin in the connecting cilium of rat photoreceptors.
The Journal of comparative neurology 1989;288(4):640-6.
1989: David S Williams; T A Shuster; M R Moldrawski; A D Blest; D B Farber
Isolation of rod outer segments on Percoll gradients: effect of specific protease inhibition.
Experimental eye research 1989;49(3):439-44.
1988: David S Williams; Kenneth A Linberg; D K Vaughan; Robert Fariss; Steven K Fisher
Disruption of microfilament organization and deregulation of disk membrane morphogenesis by cytochalasin D in rod and cone photoreceptors.
The Journal of comparative neurology 1988;272(2):161-76.
1988: Don H Anderson; David S Williams; Jay Neitz; Robert Fariss; S J Fliesler
Tunicamycin-induced degeneration in cone photoreceptors.
Visual neuroscience 1988;1(2):153-8.
1982: A D Blest; S Stowe; W Eddey; David S Williams
The local deletion of a microvillar cytoskeleton from photoreceptors of tipulid flies during membrane turnover.
Proceedings of the Royal Society of London. Series B, Containing papers of a Biological character. Royal Society (Great Britain) 1982;215(1201):469-79.
1980: A D Blest; David S Williams; L Kao
The posterior median eyes of the dinopid spider Menneus.
Cell and tissue research 1980;211(3):391-403.
1980: David S Williams; A D Blest
Extracellular shedding of photoreceptor membrane in the open rhabdom of a tipulid fly.
Cell and tissue research 1980;205(3):423-38.