Recent RPB Research & Archives

Welcome to the RPB Research Archive.  Each year, more than 800 scientific studies are published citing RPB grant support, so you should be aware that your search request may produce extensive results, requiring you to scroll through them until you locate a result of specific relevance.  You can click on any of the article titles produced by your search to find an abstract providing an overview of the research.

Search instructions
To search the entire RPB Research Archive for scientific papers on a specific topic, by a specific author, or for bibliographies by eye disease, enter keywords in the box and click Search. 

To browse bibliographies by year, click on the links below and use the search capacity within each downloaded document (Word or PDF) to find specific terms.

182 citations match:

Recent Research

A Homozygous Missense Mutation in the IRBP Gene (RBP3) Associated with Autosomal Recessive Retinitis Pigmentosa

Anneke I. den Hollander, Terri L. McGee, Carmela Ziviello, Sandro Banfi, Thaddeus P. Dryja, Federico Gonzalez-Fernandez, Debashis Ghosh, and Eliot L. Berson
Invest. Ophthalmol. Vis. Sci. 2009 50: 1864-1872.

Visual Acuity in Patients with Leber's Congenital Amaurosis and Early Childhood-Onset Retinitis Pigmentosa , 15 January 2010

Saloni Walia, Gerald A. Fishman, Samuel G. Jacobson, Tomas S. Aleman, Robert K. Koenekoop, Elias I. Traboulsi, Richard G. Weleber, Mark E. Pennesi, Elise Heon, Arlene Drack, Byron L. Lam, Rando Allikmets, Edwin M. Stone
Ophthalmology
June 2010 (Vol. 117, Issue 6, Pages 1190-1198)

Visual Acuity in Patients with Leber's Congenital Amaurosis and Early Childhood-Onset Retinitis Pigmentosa Corrected Proof, 15 January 2010

Saloni Walia, Gerald A. Fishman, Samuel G. Jacobson, Tomas S. Aleman, Robert K. Koenekoop, Elias I. Traboulsi, Richard G. Weleber, Mark E. Pennesi, Elise Heon, Arlene Drack, Byron L. Lam, Rando Allikmets, Edwin M. Stone
Ophthalmology
DOI: 10.1016/j.ophtha.2009.09.056

 

2009 Research Archive

Adaptive optics scanning laser ophthalmoscopy images demonstrate abnormal cone structure in a family with the mitochondrial DNA T8993C mutation

Yoon MK, Roorda A, Zhang Y, Nakanishi C, Wong L-JC, Zhang Q, Gillum L, Green A, Duncan JL
Invest Ophthalmol Vis Sci 2009;50:1838-1847 (Univ. of California, San Francisco - Unres. Grant, Career Development Awd and Physician-Scientist Awd) Keyword: Retinitis Pigmentosa

A homozygous missense mutation in the IRBP gene (RBP3) associated with autosomal recessive retinitis pigmentosa

den Hollander A, McGee TL, Ziviello C, Banfi S, Dryja TP, Gonzalez-Fernandez F, Ghosh D, Berson E
Invest Ophthalmol Vis Sci 2009;50(4):1864-1872 (State Univ. of New York at Buffalo – Unres. Grant) Keyword(s): Retinitis Pigmentosa

A partial structural and functional rescue of a retinitis pigmentosa model with compacted DNA nanoparticles

Cai X, Nash Z, Conley SM, Fliesler SJ, Cooper MJ, Naash MI
PLoS One 2009;4(4):e5290 (State Univ. of New York at Buffalo – Unres. Grant). Keyword(s): Gene Research/Therapy

Calcium homeostasis and cone signaling are regulated by interactions between calcium stores and plasma membrane ion channels

Szikra T, Barabas P, Bartoletti TM, Huang W, Akopian A, Thoreson WB, Krizaj D
PLOS One 2009;4(8):e6723 (Univ. of Utah - Unres. Grant) Keyword(s): Retina/Retinal Diseases, Retinitis Pigmentosa

Comparison of the new perimetric GATE strategy with conventional full-threshold and SITA standard strategies

Schiefer U, Pascual JP, Edmunds B, Feudner E, Hoffmann EM, Johnson CA, Lagrèze WA, Pfeiffer N, Sample PA, Staubach F, Weleber RG, Vonthein R, Krapp E, Paetzold J
Invest Ophthalmol Vis Sci 2009;50(1):488-494 (Oregon Health & Science Univ. – Unres. Grant) Keyword(s): Retinitis Pigmentosa, Retina/Retinal Diseases

Cytoskeletal components enhances the autophosphorylation of retinal insulin receptor

Rajala RVS, Rajala A
Chemico-Biological Interactions 2009;180:245-253 (Univ. of Oklahoma Health Sciences Center – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Diabetes reduces the autophosphorylation of retinal insulin receptor and increases protein tyrosine phosphatase-1B activity

Rajala RVS, Wiskur B, Tanito M, Callegan MC, Rajala A
Invest Ophthalmol Vis Sci 2009;50:1033-1040 (Univ. of Oklahoma Health Sciences Center – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Early synaptic defects in tulp1-/- mice

Grossman GH, Pauer GJT, Narendra U, Peachey NS, Hagstrom SA
Invest Ophthalmol Vis Sci 2009;50:3074-3083 (Cleveland Clinic Lerner College of Medicine – Unres. Grant and Sybil B. Harrington Scholar) Keyword(s): Retinitis Pigmentosa

Effect of shape and coating of a subretinal prosthesis on its integration with the retina

Butterwick A, Huie P, Jones BW, Marc RE, Marmor M, Palanker D
Exp Eye Res 2009;88(1):22-29 (Univ. of Utah - Unres. Grant and Career Development Awd) Keyword(s): Artificial Vision, Retinitis Pigmentosa

Essential and synergistic roles of RP1 and RP1L1 in rod photoreceptor axoneme and retinitis pigmentosa

Yamashita T, Liu J, Gao J, LeNoue S, Wang C, Kaminoh J, Bowne SJ, Sullivan LS, Daiger SP, Zhang K, Fitzgerald ME, Kefalov VJ, Zuo J
J. Neurosci. 2009;29:9748-60 (Washington Univ. – Challenge Grant and Career Development Awd) Keyword(s): Retina/Retinal Diseases, Retinitis Pigmentosa

Formation of all-trans retinol after visual pigment bleaching in mouse photoreceptors

Chen C, Blakeley LR, Koutalos Y
Invest Ophthalmol Vis Sci 2009;50:3589-3595 (Medical Univ. of South Carolina – Unres. Grant) Keyword(s): AMD; Retinitis Pigmentosa

Genetic heterogeneity in autosomal dominant retinitis pigmentosa with low-frequency damped electroretinographic wavelets

Aleman TS, Lam BL, Cideciyan AV, Sumaroka A, Windsor EA, Roman AJ, Schwartz SB, Stone EM, Jacobson SG
Eye (Lond) 2009;23(1):230-233 (Univ. of Miami, FL - Unres. Grant) Keyword(s): Genetics, Retinitis Pigmentosa

Genetic heterogeneity in autosomal dominant retinitis pigmentosa with low-frequency damped electroretinographic wavelets

Aleman TS, Lam BL, Cideciyan AV, Sumaroka A, Windsor EAM, Roman AJ, Schwartz SB, Stone EM, Jacobson SG
Eye 2009;23:230-3 (Univ. of Iowa - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Growth factor receptor-bound protein 14 undergoes light-dependent intracellular translocation in rod photoreceptors: Functional role in retinal insulin receptor signaling

Rajala A, Daly RJ, Tanito M, Allen DT, Holt LJ, Lobanova ES, Arshavsky VY, Rajala RVS
Biochemistry 2009;48:5563-5572 (Univ. of Oklahoma Health Sciences Center – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Harmonin in the murine retina and the retinal phenotypes of Ush1c-mutant mice and human USH1C

Williams D, Aleman TS, Lillo C, Lopes VS, Hughes LC, Stone EM, Jacobson SG
Invest Ophthalmol Vis Sci 2009;50:3881-3889 (Univ. of California, Los Angeles – Unres. Grant and Jules & Doris Stein Prof.) Keyword(s): Retina/Retinal Diseases, Retinitis Pigmentosa, Usher Syndrome

Identification of a novel palmitylation site essential for membrane association and isomerohydrolase activity of RPE65

Takahashi Y, Moiseyev G, Ablonczy Z, Chen Y, Crouch RK, Ma JX
J Biol Chem 2009;284:3211-3218 (Medical Univ. of South Carolina – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa, Retina/Retinal Diseases

Insulin receptor signaling regulates the actin cytoskeleton in developing photoreceptors

Rajala RVS, Rajala A, Brush RS, Rotstein NP, Politi LE
J Neurochem 2009;110:1648-1660 (Univ. of Oklahoma Health Sciences Center – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Mutations in SPATA7 causes Leber congenital amaurosis and juvenile retinitis pigmentosa

Wang H, den Hollander AI, Moayedi Y, Abulimiti A, Li Y, Collin RW, Hoyng CB, Lopez I, Bray M, Lewis RA, Lupski JR, Mardon G, Koenekoop RK, Chen R
Am J Human Genet 2009; 84:380-387 (Baylor College of Medicine – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Genetics

Normal cone function requires the interphotoreceptor retinoid binding protein

Parker RO, Fan J, Nickerson JM, Liou GI, Crouch RK
J Neurosci 2009;29:4616-4621 (Medical Univ. of South Carolina – Unres. Grant, Sr. Scientific Inv. Awd and Med. Student Eye Res. Fellow) Keyword(s): Retinitis Pigmentosa, Retina/Retinal Diseases

Phenotypic variability due to a novel Glu292Lys variation in exon 8 of the BEST1 gene causing Best macular dystrophy

Sohn EH, Francis PJ, Duncan JL, Weleber RG, Saperstein DA, Farrell DF, Stone EM
Arch Ophthalmol 2009;127:913-920 (Univ. of California, San Francisco - Unres. Grant, Career Development Awd and Physician-Scientist Awd) Keyword(s): Retinitis Pigmentosa, Macular Dystrophy

Predicting the pathogenicity of RPE65 mutations

Philpa AR, Jin M, Li S, Schindler EI, Iannaccone A, Lam BL, Weleber RG, Fishman GA, Jacobson SG, Mullins RF, Travis GH, Stone EM
Human Mutation 2009;30(8)1183-1188 (Univ. of Tennessee Health Science Center – Unres. Grant) Keyword(s): Gene Research/Therapy, Genetics, Retinitis Pigmentosa

Prevalence of cystic macular lesions in patients with Usher II syndrome

Walia S, Fishman GA, Hajali M
Eye (Lond) 2009;23:1206-1209 (Univ. of Illinois at Chicago - Unres. Grant) Keyword(s): Retinitis Pigmentosa, Genetics, Usher Syndrome

Remodeling of cone photoreceptor cells after rod degeneration in rd mice

Lin B, Masland RH, Strettoi E
Exper Eye Res 2009;88:589–599 (Harvard Medical School – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa.

Retinal expression of Fgf2 in RCS rats with subretinal microphotodiode array

Ciavatta VT, Kim M, Wong P, Nickerson JM, Shuler RK, McLean GY, Pardue MT
Invest Ophthalmol Vis Sci. 2009;50:4523-4530 (Emory Univ. Eye Center - Unres. Grant) Keyword(s): Retina/Retinal Diseases, Retinitis Pigmentosa

Retinal pigment epithelium defects in humans and mice with mutations in MYO7A: imaging melanosome-specific autofluorescence

Gibbs D, Cideciyan AV, Jacobson SG, Williams D
Invest Ophthalmol Vis Sci 2009;50:4386-4393 (Univ. of California, Los Angeles – Unres. Grant and Jules & Doris Stein Prof.) Keyword(s): Retina/Retinal Diseases, Retinitis Pigmentosa, Usher Syndrome

Structural Assessment of Hyperautofluorescent Ring in Patients with Retinitis Pigmentosa

Lima LH, Cella W, Greenstein VC, Wang NK, Busuioc M, Smith RT, Yannuzzi LA, Tsang SH
Retina 2009;29:1025-1031 (Columbia Univ. – Unres. Grant) Keyword(s): Retinitis Pigmentosa

The 9-methyl group of retinal is essential for rapid Meta II decay and phototransduction quenching in red cones

Estevez ME, Kolesnikov AV, Ala-Laurila P, Crouch RK, Govardovskii VI, Cornwall MC
J Gen Physiol 2009;134:137-150 (Medical Univ. of South Carolina – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa, Retina/Retinal Diseases

The action of 11-cis retinol on cone opsins and intact cone photoreceptors

Ala-Laurila P, Cornwall MC, Crouch RK, Kono M
J Biol Chem 2009;284:16492-16500 (Medical Univ. of South Carolina – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa, Retina/Retinal Diseases

The prevalence of cystoid macular oedema on optical coherence tomography in retinitis pigmentosa patients without cystic changes on fundus examination

Hajali M, Fishman GA
Eye 2009;23:915-919 (Univ. of Illinois at Chicago - Unres. Grant) Keyword(s): Retinitis Pigmentosa

The severity of retinal degeneration in Rp1H gene-targeted mice is dependent on genetic background

Liu Q, Saveliev A, Pierce EA
Invest Ophthalmol Vis Sci 2009;50:1566-1574 (Univ. of Pennsylvania – Unres. Grant) Keyword(s): Gene Therapy, Retinitis Pigmentosa

Trafficking of membrane proteins to cone but not rod outer segments is dependent on heterotrimeric kinesin-II

Avasthi P, Watt CB, Williams DS, Le YZ, Li S, Chen CK, Marc RE, Frederick JM, Baehr W
J Neurosci 2009;29(45):14287-14298 (Univ. of Utah - Unres. Grant) Keyword(s): Gene Research/Therapy, Retinitis Pigmentosa

Treatment of adult-onset acute macular retinoschisis in enhanced S-cone syndrome with oral acetazolamide

Iannaccone A, Fung KH, Eyestone ME, Stone EM
Am J Ophthalmol 2009;147(2)307-312.e2 (Univ. of Tennessee Health Science Center – Unres. Grant) Keyword(s): Gene Research/Therapy, Macular Dystrophy, Retinitis Pigmentosa, Imaging

Zeb1 represses Mift and regulates pigment synthesis, cell proliferation, and epithelial morphology

Liu Y, Ye F, Li Q, Tamiya S, Darling D, Kaplan HJ, Dean DC
Invest Ophthalmol Vis Sci 2009;50(11):5080-5088 (Univ. of Louisville – Unres. Grant.) Keyword(s): Genetics, Retinitis Pigmentosa

β-ionone activates and bleaches visual pigment in salamander photoreceptors

Isayama T, McCabe-England SL, Crouch RK, Zimmerman AL, Makino CL
Vis Neurosci 2009;26:267-274 (Medical Univ. of South Carolina – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa, Retina/Retinal Diseases

 

2008 Research Archive

11-cis and all-trans retinols can activate rod opsin: Rational design of the visual cycle

Kono M, Goletz P, Crouch RK
Biochemistry 2008;47:7567-7571 (Medical Univ. of South Carolina – Unres. Grant) Keyword(s): Retinitis Pigmentosa

A BBSome subunit links ciliogenesis, microtubule stability, and acetylation

Loktev AV, Zhang Q, Beck JS, Searby CC, Scheetz TE, Bazan JF, Slusarski DC, Sheffield VC, Jackson PK, Nachury MV
Dev Cell 2008;15:854-65 (Univ. of Iowa - Unres. Grant) Keyword(s): Retinitis Pigmentosa, Gene Therapy

Activation and membrane binding of retinal protein kinase Bα/Akt1 is regulated through light-dependent generation of phosphoinositides

Li G, Rajala A, Wichmann AF, Anderson RE, Rajala RV
J Neurochem 2008;107:1382-1397 (Univ. of Oklahoma Health Sciences Center – Unres. Grant) Keyword(s): Retinitis Pigmentosa

A high-throughput screening method for small-molecule pharmacologic chaperones of misfolded rhodopsin

Noorwez SM, Ostrov DA, McDowell JH, Krebs MP, Kaushal S
Invest Ophthalmol Vis Sci 2008;49:3224-30 (Univ. of Florida – Unres. Grant) Keyword(s): Retinitis Pigmentosa

A novel mutation and phenotypes in phosphodiesterase 6 deficiency

Tsang SH, Tsui I, Chou CL, Zernant J, Haamer E, Iranmanesh R, Tosi J, Allikmets R
Am J Ophthalmol 2008;146:780-788 (Columbia Univ. – Unres. Grant) Keyword(s): Retinitis Pigmentosa

A novel serum-free method for culturing human prenatal retinal pigment epithelial cells

Gamm DM, Melvan JN, Shearer RL, Pinilla I, Sabat G, Svendsen CN, Wright LS
Invest Ophthalmol Vis Sci 2008;49(2):788-99 (Univ. of Wisconsin – Unres. Grant and Robert E. McCormick Scholar) Keyword(s): Retinitis Pigmentosa, AMD

A pilot study of Fourier-domain optical coherence tomography of retinal dystrophy patients

Lim JI, Tan O, Fawzi AA, Hopkins JJ, Gil-Flamer JH, Huang D
Am J Ophthalmol 2008;146:417-426 (Univ. of Southern California – Unres. Grant) Keyword(s): Retinitis Pigmentosa, Retinal Dystrophy

Association of a novel mutation in the retinol dehydrogenase 12 (rdh12) gene with autosomal dominant retinitis pigmentosa

Fingert JH, Oh K, Chung M, Scheetz TE, Andorf JL, Johnson RM, Sheffield VC, Stone EM
Arch Ophthalmol 2008;126:1301-7 (Univ. of Iowa - Unres. Grant and Career Development Awd) Keyword(s): Retinitis Pigmentosa

Blockade of neuronal nitric oxide synthase reduces cone cell death in a model of retinitis pigmentosa

Komeima K, Usui S, Shen J, Rogers BS, Campochiaro PA
Free Radic Biol Med 2008;45:905-912 (Johns Hopkins Univ. – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa

Depletion of calcium stores regulates calcium influx and signal transmission in rod photoreceptors

Szikra T, Cusato K, Thoreson WB, Barabas P, Bartoletti TM, Krizaj D
J Physiol 2008;586(20):4859-4875 (Univ. of Utah - Unres. Grant) Keyword(s): Retinitis Pigmentosa, AMD

Effects of extracellular matrix and neighboring cells on induction of human embryonic stem cells into retinal or retinal pigment epithelial progenitors

Gong J, Sagiv O, Cai H, Tsang SH, Del Priore LV
Exp Eye Res 2008;86:957-965 (Columbia Univ. – Unres. Grant) Keyword(s): AMD and Retinitis Pigmentosa

Focus on molecules: Interphotoreceptor retinoid-binding protein (IRBP)

Gonzalez-Fernandez F, Ghosh D
Exp Eye Res 2008;82:169-170 (State Univ. of New York at Buffalo – Challenge Grant) Keyword(s): AMD; Retinitis Pigmentosa

Functional Rescue of Degenerating Photoreceptors in Mice Homozygous for a Hypomorphic cCMG Phosphodiesterase 6 b Allele (Pde6b H620Q)

Davis RJ, Tosi J, Janish KM, Kasanuki JM, Wang NK, Kong J, Tsui I, Cilluffo M, Woodruff ML, Fain GL, Lin CS, Tsang SH
Invest Ophthalmol Vis Sci 2008;49:5067-5076 (Columbia Univ. – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Genetic modifiers of retinal degeneration in the rd3 mouse

Danciger M, Ogando D, Yang H, Matthes MT, Yu N, Ahern K, Yasumura D, Williams RW, LaVail MM
Invest Ophthalmol Vis Sci 2008;49:2863-2869 (Univ. of California, San Francisco - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Hypoxia-regulated components of the U4/U6.U5 tri-small nuclear riboprotein complex: possible role in autosomal dominant retinitis pigmentosa

Schmidt-Kastner R, Yamamoto H, Hamasaki D, Yamamoto H, Parel JM, Schmitz C, Dorey CK, Blanks JC, Preising MN
Mol Vis 2008;14:125-135 (Univ. of Miami – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Identification of candidate genes for human retinal degeneration loci using differentially expressed genes from mouse photoreceptor dystrophy models

Demos C, Bandyopadhyay M, Rohrer B
Mol Vis 2008;14:1639-1649 (Medical Univ. of South Carolina – Unres. Grant and Olga Keith Weiss Scholar) Keyword(s): AMD, Macular Dystrophy, Retinitis Pigmentosa

Insulin growth factor 1 receptor/PI3K/AKT survival pathway in outer segment membranes of rod photoreceptors

Dilly AK, Rajala RV
Invest Ophthalmol Vis Sci 2008;49:4765-4773 (Univ. of Oklahoma Health Sciences Center – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Interaction of glyceraldehyde-3-phosphate dehydrogenase in the light-induced rod alpha-transducin translocation

Chen J, Wu M, Sezate SA, Matsumoto H, Ramsey M, McGinnis JF
J Neurochem 2008;104:1280-1292 (Univ. of Oklahoma Health Sciences Center - Unres. Grant) Keyword(s): Retinitis Pigmentosa, Retina

Ionic dysregulatory phenotyping of pathologic retinal thinning with manganese-enhanced MRI

Berkowitz BA, Gradianu M, Schafer S, Jin Y, Porchia A, Iezzi R, Roberts R
Invest Ophthalmol Vis Sci 2008;49:3178-84 (Wayne State Univ. – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Long- term cellular and regional specificity of the photoreceptor toxin, iodoacetic acid (IAA), in the rabbit retina

Liant, L, Katagirl Y, Franco LM, Yamauchi Y, Enzmann V, Kaplan HJ, Sandell JH
Vis Neuroscience 2008;25:167-177 (Univ. of Louisville –Unres. Grant) Keyword(s): Retinitis Pigmentosa

Long-term vision rescue by human neural progenitors in a rat modelof photoreceptor degeneration

Wang S, Girman S, Lu B, Bischoff N, Holmes T, Shearer R, Wright LS, Svendsen CN, Gamm DM, Lund RD
Invest Ophthalmol Vis Sci 2008;49(7):3201-6 (Univ. of Wisconsin – Unres. Grant and Robert E. McCormick Scholar) Keyword(s): Retinitis Pigmentosa

Loss of neuroprotective survival signal in mice lacking insulin receptor gene in rod photoreceptor cells

Rajala A, Tanito M, Le YZ, Kahn CR, Rajala RV
J Biol Chem 2008;283:19781-19792 (Univ. of Oklahoma Health Sciences Center – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Modulation of Phosphodiesterase6 Turnoff during Background Illumination in Mouse Rod Photoreceptors

Woodruff ML, Janisch KM, Peshenko IV, Dizhoor AM, Tsang SH, Fain GL
J Neuroscience 2008;28:2064-2074 (Columbia Univ. - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Neurturin-mediated Ret activation is required for retinal function

Brantley MA Jr, Jain S, Barr EE, Johnson EM Jr, Milbrandt J
J Neurosci 2008;28:4123-4135 (Washington Univ. – Unres. Grant). Keyword(s): Retinitis Pigmentosa

Phenotypic expression of a PRPF8 gene mutation in a large African-American family

Walia S, Fishman GA, Zernant-Rajang J, Raime K, Allikmets R
Arch Ophthalmol 2008;126:1127-1132 (Columbia Univ. – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Quantitation of the effect of hydroxylamine on rhodopsin palmitylation

Jackson W, Ablonczy Z, Crouch RK
Photochem Photobiol 2008;84:949-955 (Medical Univ. of South Carolina – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa

Regulation of photoreceptor gene expression by Crx-associated transcription factor network

Hennig AK, Peng G-H and Chen S
Brain Res 2008;1192:114-133 (Washington Univ.– Unres. Grant) Keyword(s): Retinitis Pigmentosa, Cone-rod Dystrophy

Retinal laminar architecture in human retinitis pigmentosa caused by Rhodopsin gene mutations

Aleman TS, Cideciyan AV, Sumaroka A, Windsor EAM, Herrera W, White DA, Kaushal S, Naidu A, Roman AJ, Schwartz SB, Stone EM, Jacobson SG
Invest Ophthalmol Vis Sci 2008;49:1580-1590 (Univ. of Pennsylvania – Unres. Grant) Keyword(s): Retinitis Pigmentosa, Gene Therapy

Retinal laminar architecture in human retinitis pigmentosa caused by rhodopsin gene mutations

Aleman TS, Cideciyan AV, Sumaroka A, Windsor EA, Herrera W, White DA, Kaushal S, Naidu A, Roman AJ, Schwartz SB, Stone EM, Jacobson SG
Invest Ophthalmol Vis Sci 2008;49:1580-90 (Univ. of Iowa - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Retinal laminar architecture in human retinitis pigmentosa caused by rhodopsin gene mutations

Aleman TS, Cideciyan AV, Sumaroka A, Windsor EA, Herrera W, White DA, Kaushal S, Naidu A, Roman AJ, Schwartz SB, Stone EM, Jacobson SG
Invest Ophthalmol Vis Sci 2008;49:1580-9 (Univ. of Florida – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Retinal pigment epithelium-retinal g protein receptor-opsin mediates light-dependent translocation of all-trans-retinyl esters for synthesis of visual chromophore in retinal pigment epithelial cells

Radu RA, Hu J, Peng J, Bok D, Mata NL, Travis GH
J Bio Chem 2008;283(28):19730-19738 (Univ.of California, Los Angeles – Unres. Grant and Jules & Doris Stein RPB Prof.) Keyword(s): Retinitis Pigmentosa

Retinal pigment epithelium lipofuscin proteomics

Ng KP, Gugiu B, Renganathan K, Davies MW, Gu X, Crabb JS, Kim SR, Rózanowska MB, Bonilha VL, Rayborn ME, Salomon RG, Sparrow JR, Boulton ME, Hollyfield JG, Crabb JW
Molecular & Cellular Proteomics. 2008;7(7):1397- 405 (Cleveland Clinic Lerner College of Medicine – Challenge Grant and Sr. Scientific Inv.) Keyword(s): Retinitis Pigmentosa

Retinal TrkB receptors regulate neural development in the inner, but not outer, retina

Grishanin RN, Yang H, Liu X, Donohue-Rolfe K, Nune GC, Zang K, Xu B, Duncan JL, LaVail MM, Copenhagen DR, Reichardt LF
Mol Cell Neurosci 2008;38:431-443 (Univ. of California, San Francisco – Unres. Grant, Career Development Awd, Physician Scientist Awd and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa, Low Vision

Rpe65-/- and Lrat-/- mice: comparable models of leber congenital amaurosis

Fan J, Rohrer B, Frederick JM, Baehr W, Crouch RK
IOVS 2008;49(6):2384-2389 (Univ. of Utah - Unres. Grant) Keyword(s): Leber Congential Amaurosis, Retinitis Pigmentosa

STAT3 activation in photoreceptors by leukemia inhibitory factor is associated with protection from light damage

Ueki Y, Wang J, Chollangi S, Ash JD
J of Neurochem 2008;105:784-796 (Univ. of Oklahoma Health Sciences Center - Unres. Grant) Keyword(s): AMD, Retinitis Pigmentosa

Sustained delivery of NT-3 from lens fiber cells in transgenic mice reveals specificity of neuroprotection in retinal degenerations

LaVail MM, Nishikawa S, Duncan JL, Haidong Y, Matthes MM, Yasumura D, Vollrath D, Overbeek PA, Ash JD, Robinson ML
J Comparative Neurology 2008;511(6):724-735 (Univ. of Oklahoma Health Sciences Center - Unres. Grant and Physician-Scientist Awd) Keyword(s): AMD, Retinitis Pigmentosa

Sustained delivery of NT-3 from lens fiber cells in transgenic mice reveals specificity of neuroprotection in retinal degenerations

LaVail MM, Nishikawa S, Duncan JL, Yang H, Matthes MT, Yasumura D, Vollrath D, Overbeek PA, Ash JD, Robinson ML
J Comp Neurol 2008;511:724-735 (Univ. of California, San Francisco – Unres. Grant and Physician-Scientist Awd) Keyword(s): Retinitis Pigmentosa

Tauroursodeoxycholic acid preservation of photoreceptor structure and function in the rd10 mouse through postnatal day 30

Phillips MJ, Walker TA, Choi HY, Faulkner AE, Kim MK, Sidney SS, Boyd AP, Nickerson JM, Boatright JH, Pardue MT
Invest Ophthalmol Vis Sci. 2008;49:2148-2155 (Emory Univ. Eye Center - Unres. Grant) Keyword(s): Retinitis Pigmentosa

The prevalence of cystoid macular oedema in retinitis pigmentosa patients determined by optical coherence tomography

Hajali M, Fishman GA, Anderson RJ
Br J Ophthalmol 2008;92:1065-1068 (Univ. of Illinois at Chicago - Unres. Grant) Keyword(s): Retinitis Pigmentosa

The relationship of photoreceptor degeneration to retinal vascular development and loss in mutant rhodopsin transgenic and RCS rats

Pennesi ME, Nishikawa S, Matthes MT, Yasumura D, LaVail MM
Exp Eye Res 2008;87:561-570 (Univ. of California, San Francisco - Unres. Grant) Keyword(s): Retinitis Pigmentosa, AMD

Usher syndromes due to MYO7A, PCDH15, USH2A or GPR98 mutations share retinal disease mechanism

Jacobson SG, Cideciyan AV, Aleman TS, Sumaroka A, Roman AJ, Gardner LM, Prosser HM, Mishra M, Bech-Hansen NT, Herrera W, Schwartz SB, Liu X-Z, Kimberling WJ, Steel KP, Williams DS
Human Molecular Genetics 2008;17(15):2405-2415 (Univ. of California, Los Angeles – Unres. Grant and Jules & Doris Stein RPB Prof.) Keyword(s): Retinitis Pigmentosa

 

2007 Research Archive

A novel form of transducin-dependent retinal degeneration: Accelerated retinal degeneration in the absence of rod transducin

Brill E, Malanson KM, Radu RA, Boukharov NV, Wang Z, Chung HY, Lloyd MB, Bok D, Travis GH, Obin M, Lem J
Invest Ophthalmol Vis Sci 2007;48:5445-5453 (Univ. of California, Los Angeles – Unres. Grant and Jules & Doris Stein RPB Prof.) Keyword(s): Retinitis Pigmentosa

A novel form of transducin-dependent retinal degeneration: accelerated retinal degeneration in the absence of rod transducin

Brill E, Malanson KM, Radu RA, Boukharov, NV, Wang Z, Chung H-Y, Lloyd MB, Bok D, Travis GH, Obin M, Lem J
Invest Ophthalmol Vis Sci 2007;48:5445-5453 (Tufts Univ. – Challenge Grant) Keyword(s): Retinitis Pigmentosa

Antioxidants slow photoreceptor cell death in mouse models of retinitis pigmentosa

Komeima K, Rogers BS, Campochiaro PA
J Cell Physiol 2007;213:809-815 (Johns Hopkins Univ. – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa

Apoptosis and autophagy in photoreceptors exposed to oxidative stress

Kunchithapautham K, Rohrer B
Autophagy 2007;3:433-441 (Medical Univ. of South Carolina – Unres. Grant and Olga Keith Weiss Scholar) Keyword(s): Retinitis Pigmentosa; Neurodegeneration

A strong genetic determinant of hyperoxia-related retinal degeneration on mouse chromosome 6

Smit-McBride Z, Oltjen SL, LaVail MM, Hjelmeland LM
Invest Ophthalmol Vis Sci 2007;48:405-411 (Univ. of California, San Francisco - Unres. Grant) Keyword(s): Retinitis Pigmentosa, AMD

Autophagy is one of the multiple mechanisms active in photoreceptor degeneration

Kunchithapautham K, Rohrer B
Autophagy 2007;3:65-66 (Medical Univ. of South Carolina – Unres. Grant and Olga Keith Weiss Scholar) Keyword(s): Retinitis Pigmentosa; Neurodegeneration

cAMP-responsive element binding protein mediates a cGMP/protein kinase G-dependent anti-apoptotic signal induced by nitric oxide in retinal neuro-glial progenitor cells

Nagai-Kusuhara A, Nakamura M, Mukuno H, Kanamori A, Negi A, Seigel GM
  Exp Eye Res 2007;8:152-162 (State University of New York at Buffalo – Challenge Grant and Sybil B. Harrington Scholar) Keyword(s): AMD, Retinitis Pigmentosa

Cells isolated from umbilical cord tissue rescue photoreceptors and visual functions in a rodent model of retinal disease

Lund RD, Wang S, Lu B, Girman S, Holmes T, Sauve Y, Messina DJ, Harris IR, Kihm AJ, Harmon AM, Chin F, Gosiewska A, Mistry SK
Stem Cells 2007;25:602-611 (Univ. of Utah - Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): AMD, Retinitis Pigmentosa

Chromophore switch from 11-cis-dehydroretinal (A2) to 11-cis-retinal (A1) decreases dark noise in salamander red rods

Ala-Laurila P, Donner K, Crouch RK, Cornwall MC
J Physiol 2007;585.1:57-74 (Medical Univ. of South Carolina – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa; MD

Conditional deletion of AP-2alpha in the developing retina demonstrates non-cell autonomous roles for AP-2alpha in optic cup development

Bassett EA, Pontoriero GF, Marquardt T, Fini ME, Williams T, West-Mays JA
Molecular and Cellular Biology, 2007;27:7497-7510 (Univ. of Miami – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Conserved interactions of a compact highly active enhancer/promoter upstream of the rhodopsin kinase (GRK1) gene

Young JE, Kasperek EM, Vogt TM, Lis A, Khani SC
Genomics 2007;90:236-248 (State University of New York at Buffalo – Challenge Grant) Keyword(s): AMD,Retinitis Pigmentosa

Continued use of dorzolamide for the treatment of cystoid macular oedema in patients with retinitis pigmentosa

Fishman GA, Apushkin MA
Br J Ophthalmol 2007;91:743-745 (Univ. of Illinois at Chicago - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Crx activates opsin transcription by recruiting HAT-containing co-activators and promoting histone acetylation

Peng G-H and Chen S
Hum Mol Genet 2007;16:2433-2452 (Washington Univ. - Unres. Grant and Sybil B. Harrington Scholar) Keyword(s): Retinitis Pigmentosa and Photoreceptor Degeneration

Delay of photoreceptor degeneration in tubby mouse by sulforaphane

Kong L, Tanito M, Huang Z et al.
J Neurochem 2007;101:1041-1052 (Univ. of Oklahoma – Unres. Grant) Keyword(s): Retinitis Pigmentosa, AMD

Deltion of PrBP/δ impedes transport of GRK1 and PDE6 subunits to photoreceptor outer segments

Zhang H, Li S, Doan T, Rieke F, Detwiler PB, Frederick JM, Baehr W
Proc Natl Acad Sci USA 2007;104:8857-8862 (Univ. of Utah - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Electroretinographic analyses of Rpe65-deficient mice: developing an in vivo bioassay for human gene therapy trials of Leber congenital amaurosis

Roman AJ, Boye SL, Aleman TS, Pang JJ, McDowell JH, Boye SE, Cideciyan AV, Jacobson SG, Hauswirth WW
Mol Vis 2007;13:1701-1710 (Univ. of Florida – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Engineering a "steric doorstop" in rhodopsin: converting an inverse agonist to an agonist

McKee TD, Lewis MR, Kono M
Biochemistry 2007; 46:12248-12252 (Medical Univ. of South Carolina – Unres. Grant) Keyword(s): Retinitis Pigmentosa

G-protein coupled receptor rhodopsin regulates the phosphorylation of retinal insulin receptor

Rajala A, Anderson RE, Ma JX, Lem J, Al-Ubaidi MR, Rajala RVS
  J Biol Chem 2007;282:9865-73 (Tufts Univ. – Challenge Grant) Keyword(s): Retinitis Pigmentosa

G-protein coupled receptor rhodopsin regulates the phosphorylation of retinal insulin receptor

Rajala A, Anderson RE, Ma JX, Lem J, Rajala RVS
J Biol Chem 2007;282:9865-9873 (Univ. of Oklahoma - Unres. Grant and Career Development Award) Keyword(s): Retinitis Pigmentosa.

High-resolution in vivo imaging of the rpe mosaic in eyes with retinal disease


Roorda A, Zhang Y, Duncan J
Invest Ophthalmol Vis Sci 2007;48:2297-2303 (Univ. of California, San Francisco – Unres. Grant and Career Development Awd) Keyword(s): Retinitis Pigmentosa

High-speed, ultrahigh resolution optical coherence tomography of the retina in hunter syndrome

Yoon MK, Chen RW, Hedges TR, Srinivasan VJ, Gorczynska I, Fujimoto JG, Wojtkowski M, Schuman JS, Duker JS
Ophthalmic Surg Lasers Imaging 2007;38:423-428 (Tufts Univ. – Challenge Grant and Medical Student Fellowship) Keyword(s): Retinitis Pigmentosa and Diabetic Retinopathy

High resolution imaging with adaptive optics in patients with inherited retinal degeneration

Duncan JL, Zhang Y, Gandhi J, Nakanishi C, Othman M, Branham KEH, Swaroop A, Roorda A
Invest Ophthalmol Vis Sci 2007;48:3283-3291 (Univ. of California, San Francisco – Unres. Grant and Career Development Awd) Keyword(s): Retinitis Pigmentosa

Improved retinal transduction in vivo and photoreceptor-specific transgene expression using adenovirus with modified penton base

Cashman SM, McCullough L, Kumar-Singh K
Mol Ther 2007;15:1640-1646 (Tufts Univ. – Challenge Grant) Keyword(s): Retinitis Pigmentosa

Interaction between the photoreceptor-specific tubby-like protein 1 and the neuronal –specific GTPase dynamin-1

Xi Q, Pauer GJT, Ball SL, Rayborn M, Hollyfield JG, Peachey NS, Crabb JW, Hagstrom SA
Invest Ophthalmol Vis Sci 2007;48:2837-2844 (Cleveland Clinic Lerner College of Medicine – Challenge Grant) Keyword(s): Retinitis Pigmentosa

Interphotoreceptor retinoid-binding protein is the physiologically relevant carrier that removes retinol from rod photoreceptor outer segments

Wu Q, Blakeley LR, Cornwall MC, Crouch RK, Wiggert BN, Koutalos Y
Biochemistry 2007;46:8669-8679 (Medical Univ. of South Carolina – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): AMD, retinitis pigmentosa

Intersession repeatability of humphrey perimetry measurements in patients with retinitis pigmentosa

Kim LS, McAnany JJ, Alexander KR, Fishman GA
Invest Ophthalmol Vis Sci 2007;48:4720-4724 (Univ. of Illinois at Chicago - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Intracellular organelles and calcium homeostatis in rods and cones

Szikra T, Krizaj D
Vis Neurosci 2007;24:733-743 (Univ. of Utah - Unres. Grant and James S. Adams Scholar) Keyword(s): Retinitis Pigmentosa, Glaucoma

Intraocular CNTF reduces vision in normal rats in a dose-dependent manner

McGill TJ, Prusky GT, Douglas RM, Yasumura D, Matthes MT, Nune G, Donohue-Rolfe K, Yang H, Niculescu D, Hauswirth WW, Girman SV, Lund RD, Duncan JL, LaVail MM
Invest Ophthalmol Vis Sci 2007;48:5756-5766 (Univ. of California, San Francisco - Unres. Grant) Keyword(s): Retinitis Pigmentosa, AMD

IRE1 signaling affects cell fate during the unfolded protein response

Lin JH, Li H, Yasumura D, Cohen HR, Zhang C, Panning B, Shokat KM, LaVail MM, Walter P
Science 2007;318:944-949 (Univ. of California, San Francisco - Unres. Grant) Keyword(s): Retinitis Pigmentosa, AMD

Isoflurane is an effective alternative to ketamine/xylazine/acepromazine as an anesthetic agent for the mouse electroretinogram

Woodward WR, Choi D, Grose J, Malmin B, Hurst S, Pang J, Weleber RG, Pillers DA
Doc Ophthalmol 2007;115:187-201 (Oregon Health & Science Univ. – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Melanopsin-dependent persistence and photopotentiation of murine pupillary light responses

Zhu Y, Tu DC, Denner D, Shane T, Fitzgerald CM, Van Gelder RN
Invest Ophthalmol Vis Sci 2007;48:1268-1275 (Washington Univ. - Unres. Grant and Career Development Awd) Keyword(s): Low Vision, Retinitis Pigmentosa

Module structure of interphotoreceptor retinoid-binding protein (IRBP) may provide bases for its complex role in the visual cycle - structure / function study of Xenopus IRBP

Gonzalez-Fernandez F, Baer CA, Ghosh D
BMC Biochem 2007;8:15. (State University of New York at Buffalo – Challenge Grant) Keyword(s): AMD, Retinitis Pigmentosa

Molecular and cellular alterations induced by chronic expression of ciliary neurotrophic factor in a mouse model of retinitis pigmentosa

Rhee DR, Ruiz A, Duncan JL, Hauswirth WW, LaVail MM, Bok D, Yang X-J
Invest Ophthalmol Vis Sci;2007;48:1389-1400 (Univ. of Florida – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Molecular and cellular alterations induced by sustained expression of ciliary neurotrophic factor in a mouse model of retinitis pigmentosa

Rhee KD, Ruiz A, Duncan JL, Hauswirth WW, LaVail MM, Bok D, Yang X-J
Invest Ophthalmol Vis Sci 2007;48:1389-1400 (Univ. of California, San Francisco - Unres. Grant) Keyword(s): Retinitis Pigmentosa, AMD

Molecular and cellular alterations induced by sustained expression of ciliary neurotrophic factor in a mouse model of retinitis pigmentosa

Rhee KD, Ruiz A, Duncan, JL, Hauswirth WW, LaVail MM, Bok D, Yang XJ
Invest Ophthalmol Vis Sci 2007;48:1389-1400 (Univ. of California, Los Angeles – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Natural course of visual field loss in patients with type 2 usher syndrome

Fishman GA, Bozbeyoglu S, Massof RW, Kimberling W
Retina 2007; 27:601-608 (Univ. of Illinois at Chicago - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Neural reprogramming in retinal degeneration

Marc RE, Jones BW, Anderson JR, Kinard K, Marshak DW, Wilson JH, Wensel T, Lucas RJ
Invest Ophthalmol Vis Sci 2007;48:3364-3371 (Univ. of Utah - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Nonredundant role of Akt2 for neuroprotection of rod photoreceptor cells from light-induced cell death

Li G, Anderson RE, Tomita H, Adler R, Liu X, Zack DJ, Rajala RVS
The J. Neurosci 2007;27:203-211 (Univ. of Oklahoma – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Paradoxical role of BDNF: BDNF+/- retinas are protected against light damage-mediated stress

Wilson RB, Kunchithapautham K, Rohrer B
Invest Ophthalmol Vis Sci 2007;48:2877-2886 (Medical Univ. of South Carolina – Unres. Grant and Olga Keith Weiss Scholar) Keyword(s): Retinitis Pigmentosa; Neuroprotection

Phosducin regulates the expression of transducin βγ subunits in rod photoreceptors and does not contribute to phototransduction adaptation

Krispel CM, Sokolov M, Chen WM, Song H, Herrmann R, Arshavsky VY, Burns ME
J Gen Physiol 2007;130:303-312 (Duke Univ. – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis pigmentosa

Preservation of outer retina and its synaptic connectivity following subretinal injections of human RPE cells in the royal college of surgeons rat

Pinilla I, Cuenca N, Sauve Y, Wang S, Lund RD
Exp Eye Res 2007;85:381-392 (Univ. of Utah - Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): AMD, Retinitis Pigmentosa

Protection of visual functions by human neural progenitors in a rat model of retinal disease

Gamm DM, Wang S, Lu B, Girman S, Holmes T, Bischoff N, Shearer RL, Sauvé Y, Capowski E, Svendsen CN, Lund RD
PLoS ONE 2007;3:e338 (Univ. of Wisconsin – Unres. Grant and Robert E. McCormick Scholar) Keyword(s): Retinitis Pigmentosa, AMD

Protection of visual functions by human neural progenitors in a rat model of retinal disease

Gamm DM, Wang S, Girman S, Holmes T, Bischoff N, Shearer RL, Sauve Y, Capowski E, Svendsen CN, Lund RD
PLOS Med 2007;3:e338 (Univ. of Utah - Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): AMD, Retinitis Pigmentosa

Purification of the full-length Xenopus interphotoreceptor retinoid binding protein and growth of diffraction-quality crystals

Ghosh D, Griswold JB, Bevilacqua T, Gonzalez-Fernandez F
Mol Vis 2007;13:2275-2281 (State University of New York at Buffalo – Challenge Grant) Keyword(s): AMD,Retinitis Pigmentosa

Quantitative genetics of age-related retinal degeneration: a second F1 intercross between the A/J and C57BL/6 strains

Danciger M, Yang H, Ralston R, Liu Y, Matthes MT, Peirce J, Lavail MM
Mol Vis 2007;13:79-85 (Univ. of California, San Francisco - Unres. Grant) Keyword(s): Retinitis Pigmentosa, AMD

Rapid and stable knockdown of an endogenous gene in retinal pigment epithelium

Paskowitz DM, Greenberg KP, Yasumura D, Grimm D, Yang H, Duncan JL, Kay MA, LaVail MM, Flannery JG, Vollrath D
Hum Gene Ther 2007;18:871-880 (Univ. of California, San Francisco - Unres. Grant) Keyword(s): Retinitis Pigmentosa, AMD

Rebound of cystoid macular edema with continued use of acetazolamide in patients with retinitis pigmentosa

Apushkin MA, Fishman GA, Grover S, Janowicz MJ
Retina 2007;27:1112-1118 (Univ. of Illinois at Chicago - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Retinal thickness and visual thresholds measured in patients with retinitis pigmentosa

Apushkin MA, Fishman GA, Alexander KR, Shahidi M
Retina 2007;27:349-357 (Univ. of Illinois at Chicago - Unres. Grant) Keyword(s): Retinitis Pigmentosa.

SARA-regulated vesicular targeting underlies formation of the light sensing organelle in mammalian rods

Chuang J-Z, Zhao Y, Sung C-H
Cell 130:535-547 (Weill Cornell Medical College - Challenge Grant) Keyword(s): Retinal Degeneration, Retinitis Pigmentosa

Senseless functions as a molecular switch for color photoreceptor differentiation in Drosophila

Xie B, McDonald E, Gebelein B, Cook T
Development 2007;134:4243-4253 (Univ. of Cincinnati – Unres. Grant) Keyword(s): Gene Therapy, Retinitis Pigmentosa

Simultaneous fundus imaging and optical coherence tomography of the mouse retina

Kocaoglu OP, Uhlhorn SR, Hernandez E, Juarez RA, Will R, Parel JM, Manns F
Invest Ophthalmol Vis Sci 2007;48:1283-9 (Univ. of Miami – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Symptomatic posterior vitreous detachment and the incidence of delayed retinal breaks – case series and meta-analysis

Coffee RE, Westfall AC, Davis GH, Mieler WF, Holz ER
Am J Ophthalmol 2007;144:409-413 (Baylor College of Medicine – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Syngeneic schwann cell transplantation preserves vision in RCS rat without immunosuppresion

McGill TJ, Lund RD, Douglas RM, Wang S, Lu B, Silver BD, Secretan MR, Arthur JN, Prusky GT
Invest Ophthalmol Vis Sci 2007;48:1906-1912 (Univ. of Utah - Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): AMD, Retinitis Pigmentosa

The coexistence of age-related macular degeneration and retinitis pigmentosa in three unrelated families

Sarraf D, Salib DM, Jain A, Quiram PA
Semin Ophthalmol 2007;22:155-161 (Univ. of California, Los Angeles - Unres. Grant) Keyword(s): ARMD, Retinitis Pigmentosa

The function of guanylate cyclase 1 and guanylate cyclase 2 in rod and cone photoreceptors

Baehr W, Karan S, Maeda T, Luo D, Li S, Bronson D, Watt CB, Yau K, Frederick JM, Palczewski K
J Biol Chem 2007;282:8837-8847 (Univ. of Utah - Unres. Grant) Keyword(s): Retinitis Pigmentosa, Cone Dystrophy

Transducin translocation in rods is triggered by saturation of the GTPase activating complex

Lobanova ES, Finkelstein S, Song H, Tsang SH, Chen C-K, Sokolov M, Skiba NP, Arshavsky VY
J Neurosci 2007;27:1151–1160 (Duke Univ. – Unres. Grant and Sr. Scientific Inv. Awd): Keyword(s): Retinitis Pigmentosa

Two mouse retinal degenerations caused by missense mutations in the beta-subunit of rod cGMP phosphodiesterase gene

Chang B, Hawes NL, Pardue MT, German AM, Hurd RE, Davisson MT, Nusinowitz S, Rengarajan K, Boyd AP, Sidney SS, Phillips MJ, Stewart RE, Chaudhury R, Nickerson JM, Heckenlively JR, Boatright JH
Vision Res. 2007;47:624-33 (Univ. of Michigan – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Visual performance using a retinal prosthesis in three subjects with retinitis pigmentosa

Yanai D, Weiland JD, Mahadevappa M, Greenberg RJ, Fine I, Humayun MS
Am J Ophthalmol 2007;143:820-827 (Univ. of Southern California – Unres. Grant) Keyword(s): Artificial Vision, Retinitis Pigmentosa

 

2006 Research Archive

Activation Phase of Cone Phototransduction and the Flicker Electroretinogram in Retinitis Pigmentosa

Alexander KR, Rajagopalan AS, Raghuram A, Fishman GA
Vision Research 2006;46:2773-85 (Univ. of Illinois at Chicago – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Alternate Binding Mode of C-terminal Phenethylamine Analogs of Gtα(340-350) to Photoactivated Rhodopsin

Anderson MA, Ogbay B, Kisselev OG, Cistola DP, Marshall GR
Chem Biol Drug Des 2006;68:295-307 (Saint Louis Univ. – Challenge Grant and Wm and Mary Greve Scholar) Keyword(s): Retinitis Pigmentosa

A Novel RPGR Exon ORF15 Mutation in a Family with X-linked Retinitis Pigmentosa and Coats'-Like exudative Vasculopathy

Demirci FY, Rigatti BW, Mah TS, Gorin MB
Am J Ophthalmol 2006;141:208-210 (Univ. of Pittsburgh – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinal Vessels; Retinitis Pigmentosa

Bright Cyclic Light Accelerates Photoreceptor Cell Degeneration in Tubby Mice

Kong L, Li F, Soliman CE, Li S, Elias RV, Zhou X, Lewis DA, McGinnis JF, Cao W
Neurobiol Dis 2006;21:468-477 (Univ. of Oklahoma Health Sciences Center ― Unres. Grant) Keyword(s): Retinitis Pigmentosa

Clinical Features Of Autosomal Dominant Retinitis Pigmentosa Associated With A Rhodopsin Mutation

Chen H, Chen Y, Horn R, Yang Z, Wang C, Turner MJ, Zhang K
Ann Acad Med 2006;35(6):411-415 (Univ. of Utah - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Engrafted Neural Progenitor Cells Express a Tissue-Restricted Reporter Gene Associated with Differentiated Retinal Photoreceptor Cells

Sam TN, Xiao J, Roehrich H, Low WC, Gregerson DS
Cell Transplant 2006;15:147-160 (Univ. of Minnesota – Unres. Grant) Keyword(s): AMD and Retinitis Pigmentosa

G-protein Alpha and Beta-Gamma Subunits Interact with Conformationally Distinct Signaling States of Rhodopsin

Downs MA, Arimoto R, Marshall GR, Kisselev OG
Vis Res 2006;46:4442-4448 (Saint Louis Univ. – Challenge Grant and Wm. and Mary Greve Scholar) Keyword(s): Retinitis Pigmentosa

GAP-Independent Termination of Photoreceptor Light Response by Excess γ Subunit of the cGMP-Phosphodiesterase

Tsang SH, Woodruff ML, Chen CK, Yamashita CY, Cilluffo MC, Rao AL, Farber DB, Fain GL
J Neurosci 2006; 26:4472-4480 (Columbia Univ. – Unres. Grant) Keyword(s) Retinitis Pigmentosa

Genomic Rearrangements of the PRPF31 Gene Account for 2

Sullivan LS, Bowne SJ, Seaman CR, Blanton SH, Lewis RA, Heckenlively JR, Birch DG, Hughbanks-Wheaton D, Daiger SP
5% of Autosomal Dominant Retinitis Pigmentosa. Invest Ophthal Vis Sci 2006;47:4579-4588 (Baylor College of Medicine – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa

Implantation of an Inactive Epiretinal Poly(Dimethyl Siloxane) Electrode Array in Dogs

Guven D, Weiland JD, Maghribi M, Davidson JC, Mahadevappa M, Roizenblatt R, Qiu G, Krulevitz P, Wang X, Labree L, Humayun MS
Exp Eye Res 2006;82:81-90 (Univ. of Southern California – Unres. Grant) Keyword(s): Artificial Vision, Retinitis Pigmentosa

Inner Retinal Mechanisms Engaged by Retinal Electrical Stimulation

Margalit E, Thoreson WB
Invest Ophthalmol Vis Sci 2006;47:2606-2612 (Univ. of Nebraska Medical Center – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Inner Retinal Photoreception Independent of the Visual Retinoid Cycle

Tu DC, Owens LA, Anderson L, Golczak M, Doyle SE, McCall M, Menaker M, Palczewski K, Van Gelder RN
Proc Natl Acad Sci U S A 2006;103(27):10426-31 (Washington Univ. – Unres. Grant and Career Development Awd) Keyword(s): Retinitis Pigmentosa, Gene Therapy

Intavitreal Triamcinolone Acetonide for Treatment of Cystoid Macular Edema Associated with Retinitis Pigmentosa

Kim JE
Retina 2006;26:1094-1096 (Medical College of Wisconsin – Unres. Grant) Keyword(s): Retinal and Retinal Diseases

Light and Inherited Retinal Degeneration

Paskowitz DM, LaVail MM, Duncan JL
Br J Ophthalmol 2006;90:1060-6 (Univ. of California, San Francisco – Unres. Grant and Career Development Awd) Keyword(s): AMD, Retinitis Pigmentosa

Localization Of The Insulin Receptor And Phosphoinositide 3-Kinase In Detergent-Resistant Membrane Rafts Of Rod Photoreceptor Outer Segments

Rajala RVS, Elliott MH, McClellan ME, Anderson RE
Advances in Experimental Medicine and Biology 2006;572: 491-497 (Univ. of Oklahoma, Oklahoma City – Unres. Grant and Career Development Awd) Keyword(s): Retinitis Pigmentosa

Prevalence of Disease-Causing Mutations in Families with Autosomal Dominant Retinitis Pigmentosa: A Screen of Known Genes in 200 Families

Sullivan LS, Bowne SJ, Birch DG, Hughbanks-Wheaton D, Heckenlively JR, Lewis RA, Garcia CA, Ruiz RS, Blanton SH, Horthrup H, Gire AI, Seaman R, Duzkale H, Spellicy CJ, Zhu J, Shankar SP, Daiger SP
Invest Ophthal Vis Sci 2006;47:3052-3064 (Baylor College of Medicine – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa

Regulatory Dissociation Of Tctex-1 Light Chain From Dynein Complex Is Essential For The Apical Delivery Of Rhodopsin

Yeh TY, Peretti D, Chuang JZ, Rodriguez-Boulan E, Sung CH
Traffic 2006;7(11):1495-1502 (Weill-Cornell Univ. – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Relative Strength of Cation-π vs Salt-Bridge Interactions: The Gtα(340–350) Peptide/Rhodopsin System

Anderson MA, Ogbay B, Arimoto R, Sha W, Kisselev OG, Cistola DP, Marshall GR
J Am Chem Soc 2006;128:7531-7541 (Saint Louis Univ. - Challenge Grant and Wm. and Mary Greve Scholar) Keyword(s): Retinitis Pigmentosa

Rhodopsin-Interacting Surface of the Transducin γ Subunit

Kisselev O, Downs M
Biochem J 2006;45:9386-9392 (Saint Louis Univ. – Challenge Grant and Wm. and Mary Greve Scholar) Keyword(s): Retinitis Pigmentosa

Scotopic Visual Signaling in the Mouse Retina is Modulated by High Affinity Plasma Membrane Calcium Extrusion

Duncan JL, Yang H, Doan T, Silverstein B, Murphy GJ, Nune G, Liu X, Copenhagen DR, Rieke F, Tempel BL, Krizaj D
J Neurosci 2006;26:7201-11 (Univ. of California, San Francisco – Unres. Grant, Career Development Awd and James S. Adams Scholar ) Keyword(s): Retinitis Pigmentosa

Spectrum and Frequency of Mutations in IMPDH1 Associated with Autosomal Dominant Retinitis Pigmentosa and Leber Congenital Amaurosis

Bowne SJ, Sullivan LS, Mortimer SE, Hedstrom L, Zhu J, Spellicy CJ, Gire AI, Hughbanks-Wheaton D, Birch DG, Lewis RA, Heckenlively JR, Daiger SP
Invest Ophthal Vis Sci 2006;47:34-42 (Baylor College of Medicine – Unres. Grant and Sr. Scientific Inv. Awd) Keyword(s): Retinitis Pigmentosa

The Dynamic Range and Domain-Specific Signals of Intracellular Calcium in Photoreceptors

Szikra T, Krizaj D.
Neuroscience 2006;141:143-155 (Univ. of California, San Francisco – Unres. Grant, James S. Adams Scholar Awd) Keyword(s): Retinitis Pigmentosa

Topical Dorzolamide for the Treatment of Cystoid Macular Edema in Patients with Retinitis Pigmentosa

Grover S, Apushkin MA, Fishman GA
Am J Ophthalmol 2006;141:850-8 (Univ. of Illinois at Chicago - Unres. Grant) Keyword(s): Retinitis Pigmentosa

Transgenic Mice Carrying the H258N Mutation in the Gene Encoding the β-Subunit of Phosphodiesterase-6 (PDE6B) Provide a Model for Human Congenital Stationary Night Blindness

Tsang SH, Woodruff ML, Lin J, Mahajan V, Yamashita CK, Pedersen R, Lin CS, Goff SP, Rosenberg T, Larsen M, Farber DB and Nusinowitz S
Human Mutation 2006;0:1-12 (Columbia Univ. – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Truncation Mutation In HRG4 (UNC119) Leads To Mitochondrial ANT-1-Mediated Photoreceptor Synaptic And Retinal Degeneration By Apoptosis

Mori N, Ishiba Y, Kubota S, Kobayashi A, Higashide T, McLaren MJ, Inana G
Invest Ophthalmol Vis Sci 2006;47(4):1281-92 (Univ. of Miami – Unres. Grant) Keyword(s): Retinitis Pigmentosa

 

2005 Research Archive

A Novel GCAP1 Missense Mutation (L151F) in a Large Family with Autosomal Dominant Cone-Rod Dystrophy (adCORD)

Sokal I, Dupps WJ, Grassi MA, Brown J, Affatigato LM, Roychowdhury N, Yang L, Filipek S, Palczewski K, Stone EM, Baehr W
Invest Ophthalmol Vis Sci 2005;46:1124-1132 (Univ. of Utah, Univ. of Washington and Univ. of Iowa - Unres. Grants) Keyword(s): Retina and Retinal Diseases, Retinitis Pigmentosa

Autosomal Dominant Cone Dystrophy Caused by a Novel Mutation in the GCAP1 Gene (GUCA1A)

Jiang L, Katz BJ, Yang Z, Zhao Y, Faulkner N, Hu J, Baird J, Baehr W, Creel DJ, Zhang K
Mol Vis 2005;11:143-151 (Univ. of Utah - Unres. Grant) Keyword(s): Retina and Retinal Diseases, Retinitis Pigmentosa

Contrast Response Properties of Magnocellular and Parvocellular Pathways in Retinitis Pigmentosa Assessed by the Visual Evoked Potential

Alexander KR, Rajagopalan AS, Seiple W, Zemon VM, Fishman GA
Invest Ophthalmol Vis Sci 2005;46:2967-2973 (Univ. of Illinois at Chicago – Unres. Grant.) Keyword(s): Retinitis Pigmentosa

Deficiency of Panthothenate Kinase 2 (Pank2) in Mice Leads to Retinal Degeneration and Azoospermia

Kuo YM, Duncan JL, Westaway SK, Yang H, Nune G, Xu EY, Hayflick SJ, Gitschier J
Hum Mol Genet 2005;14:49-57 (Univ. of California, San Francisco - Unres. Grant) Keyword(s): Retinitis Pigmentosa, Retina and Retinal Diseases

Disease Expression in Usher Syndrome Caused by VLGR1 Gene Mutation (USH2C) and Comparison with USH2A Phenotype

Schwartz SB, Aleman TX, Cideciyan AV, Windsor EAM, Sumaroka A, Roman AJ, Rane T, Smilko EE, Bennett J, Stone EM, Kimberling WJ, Liu X-Z, Jacobson SG
Invest Ophtha Vis Sci 2005;46:734-743 (Univ. of Pennsylvania and Univ. of Iowa – Unres. Grants) Keyword(s): Retinitis Pigmentosa

Enhanced Cone Dysfunction in Rats Homozygous for the P23H Rhodopsin Mutation

Pinilla I, Lund RD, Sauve Y
Neurosci Lett 2005;283:16-21 (Univ. of Utah - Unres. Grant) Keyword(s): Retinitis Pigmentosa, Other: ERG

Excitation Mapping with the Organic Cation AGB2+

Marc, RE, Kalloniatis M, Jones BW
Vision Res 2005;45:3454-3468 (Univ. of Utah - Unres. Grant) Keyword(s): Retina and Retinal Diseases, Retinitis Pigmentosa

Focus on Molecules: Cellular Retinaldehyde-Binding Protein (CRALBP)

Saari JC, Crabb JW
Exp Eye Res 2005;81:245-246 (Univ. of Washington, Seattle - Unres. Grant) Keyword(s): Retinitis Pigmentosa; AMD

Focus on Molecules: Rhodopsin

Kisselev OG
Exp Eye Res 2005;81:366-367 ((Saint Louis Univ. – Challenge Grant) Keyword(s): Retina and Retinal Diseases, Retinitis Pigmentosa, Other: Phototransduction

Glutamate Transport by Retinal Muller Cells in Glutamate/Aspartate Transporter- Knockout Mice

Sarthy VP, Pignataro L, Pannicke T, Weick M, Reichenbach A, Harada T, Tanaka K, Marc R
GLIA 2005;48:184-196 (Univ. of Utah and Northwestern Univ. - Unres. Grants) Keyword(s): Retina and Retinal Diseases, Retinitis Pigmentosa

Higher-Order Wavefront Aberrations in Retinitis Pigmentosa

Rajagopalan AS, Shahidi M, Alexander KR, Fishman GA, Zelkha R
Optom Vis Sci 2005;82:623-628 (Univ. of Illinois at Chicago - Unres. Grant) Keyword(s): Retinitis Pigmentosa; Cataract

Identification of a NPXY Motif in Growth Factor Receptor Bound Protein-14 and its Interaction with the Phosphotyrosine Binding Domain of IRS-1

Rajala RVS, Chan MD
Biochem 2005;44:7929-7935 (Univ. of Oklahoma – Unres. Grant and Career Development Awd.) Keyword(s): Retinitis Pigmentosa

Insights into X-Linked Retinitis Pigmentosa Type 3, Allied Diseases and Underlying Pathomechanisms

Ferreira PA
Hum Mol Gene 2005;14:R259-R267 (Duke Univ. – Unres. Grant and Jules & Doris Stein RPB Prof ) Keyword(s): Retinal and Retinal Diseases

In Vivo Dynamics of Retinal Injury and Repair I the Rhodopsin Mutant Dog Model of Human Retinitis Pigmentosa

Cideciyan AF, Jacobson SG, Aleman TS, Gu D, Pearce-Kelling SE, Sumaroka A, Ackland GM, Aguirre GD
PNAS 2005;102:5233-5238 (Univ. of Pennsylvania – Unres. Grant) Keyword(s): Gene Therapy, Retina and Retinal Diseases

Multifocal Visual Evoked Potentials to Cone Specific Stimuli in Patients with Retinitis Pigmentosa

Holopigian K, Shuwairi SM, Greenstein VC, Winn BJ, Zhang X, Carr RE, Hood DC
Vis Res 2005;45:3244-3252 (Columbia Univ. – Unres. Grant) Keyword(s): Color Vision, Retinitis Pigmentosa, Multifocal ERG, Multifocal VEP, Visual Fields

Mutant Carbonic Anhydrase 4 Impairs pH Regulation and Causes Retinal Photoreceptor Degeneration

Yang Z, Alvarez BV, Chakarova C, Jiang L, Karan G, Frederick JM, Zhao Y, Sauve Y, Li X, Zrenner E, Wissinger B, Den Hollander AI, Katz B, Baehr W, Cremers FP, Casey JR, Bhattacharya SS, Zhang K
Hum Mol Genet 2005;14:255-265 (Univ. of Utah - Unres. Grant) Keyword(s): Retina and Retinal Diseases, Retinitis Pigmentosa

Oxidative Damage is a Potential Cause of Cone Cell Death in Retinitis Pigmentosa

Shen J, Yang X, Dong A, Petters RM, Peng YW, Wong F, Campochiaro PA
J Cell Physiol 2005;203:457-464 (Johns Hopkins Univ. and Duke Univ. – Unres. Grants and Lew R. Wasserman Merit Awd.) Keyword(s): Retinitis Pigmentosa

Quantifying Rod Photoreceptor-mediated Vision in Retinal Degenerations: Dark-Adapted Thresholds as Outcome Measures

Roman AJ, Schwartz SB, Aleman TX, Cideciyan AV, Chico JD, Windsor EAM, Gardner LM, Ying G-Y, Smilko EE, Maguire MG, Jacobson SG
Exp Eye Res 2005;80:259-272 (Univ. of Pennsylvania – Unres. Grant) Keyword(s): Retinitis Pigmentosa

Retinal Remodeling During Retinal Degeneration

Jones BW, Marc RE
Exp Eye Res 2005;81:123-137 (Univ. of Utah - Unres. Grant) Keyword(s): Retina and Retinal Diseases, Retinitis Pigmentosa

RPGR-ORF15, Which is Mutated in Retinitis Pigmentosa, Associates with SMC1, SMC3, and Microtubule Transport Proteins

Khanna H, Hurd TW, Lillo C, Shu X, Parapuram SK, He S, Akimoto M, Wright AF, Margolis B, Williams DS, Swaroop A
J Biol Chem 2005;280:33580-33587 (Univ. of Michigan – Unres. Grant and Sr. Scientific Inv. Awd.) Keyword(s): Retinal and Retinal Diseases

The Spread of Apoptosis Through Gap-Junctional Channels in BHK Cells Transfected with Cx32

Udawatte C, Ripps H
Apoptosis 2005;10:1019-1029 (Univ. of Illinois at Chicago - Unres. Grant and Sr. Scientivic Inv. Awd.) Keyword(s): Retinitis Pigmentosa