Fulton Wong

Journal Article Biochim Biophys Acta Mol Basis Dis · October 1, 2019 Leber congenital amaurosis (LCA) is the most serious form of inherited retinal dystrophy that leads to blindness or severe visual impairment within a few months after birth. Approximately 1-2% of the reported cases are caused by mutations in the LCA5 gene. ... Full text Link to item Cite

Deletion of the transmembrane protein Prom1b in zebrafish disrupts outer-segment morphogenesis and causes photoreceptor degeneration.

Journal Article J Biol Chem · September 20, 2019 Mutations in human prominin 1 (PROM1), encoding a transmembrane glycoprotein localized mainly to plasma membrane protrusions, have been reported to cause retinitis pigmentosa, macular degeneration, and cone-rod dystrophy. Although the structural role of PR ... Full text Link to item Cite

Knockout of Nr2e3 prevents rod photoreceptor differentiation and leads to selective L-/M-cone photoreceptor degeneration in zebrafish.

Journal Article Biochim Biophys Acta Mol Basis Dis · June 1, 2019 Mutations in the photoreceptor cell-specific nuclear receptor gene Nr2e3 increased the number of S-cone photoreceptors in human and murine retinas and led to retinal degeneration that involved photoreceptor and non-photoreceptor cells. The mechanisms under ... Full text Link to item Cite

CERKL gene knockout disturbs photoreceptor outer segment phagocytosis and causes rod-cone dystrophy in zebrafish.

Journal Article Hum Mol Genet · June 15, 2017 In humans, CERKL mutations cause widespread retinal degeneration: early dysfunction and loss of rod and cone photoreceptors in the outer retina and, progressively, death of cells in the inner retina. Despite intensive efforts, the function of CERKL remains ... Full text Link to item Cite

The Survival of Cone Photoreceptors in Retinitis Pigmentosa.

Journal Article JAMA Ophthalmol · March 2016 Full text Link to item Cite

A novel rhodopsin point mutation, proline-170-histidine, associated with sectoral retinitis pigmentosa.

Journal Article Ophthalmic Genet · December 2014 Identification and classification of all retinitis pigmentosa (RP) causing mutations contribute to a better understanding of disease variants. In this report we describe a New Zealand family, of European heritage, affected by a sectoral type RP phenotype i ... Full text Link to item Cite

CERKL interacts with mitochondrial TRX2 and protects retinal cells from oxidative stress-induced apoptosis.

Journal Article Biochim Biophys Acta · July 2014 Mutations in the ceramide kinase-like gene (CERKL) are associated with severe retinal degeneration. However, the exact function of the encoded protein (CERKL) remains unknown. Here we show that CERKL interacts with mitochondrial thioredoxin 2 (TRX2) and ma ... Full text Link to item Cite

Does retinitis pigmentosa cause depression?

Conference INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · June 1, 2013 Link to item Cite

Rhodopsin F45L Allele Does Not Cause Autosomal Dominant Retinitis Pigmentosa in a Large Caucasian Family.

Journal Article Transl Vis Sci Technol · February 2013 PURPOSE: To ascertain the potential pathogenicity of a retinitis pigmentosa (RP)-causing RHO F45L allele in a family affected by congenital achromatopsia (ACHM). METHODS: Case series/observational study that included two patients with ACHM and 24 extended ... Full text Link to item Cite

Novel compound heterozygous mutations in MYO7A in a Chinese family with Usher syndrome type 1.

Journal Article Mol Vis · 2013 PURPOSE: To identify the disease-causing mutation(s) in a Chinese family with autosomal recessive Usher syndrome type 1 (USH1). METHODS: An ophthalmic examination and an audiometric test were conducted to ascertain the phenotype of two affected siblings. T ... Link to item Cite

Photoreceptor Differentiation following Transplantation of Allogeneic Retinal Progenitor Cells to the Dystrophic Rhodopsin Pro347Leu Transgenic Pig.

Journal Article Stem Cells Int · 2012 Purpose. Transplantation of stem, progenitor, or precursor cells has resulted in photoreceptor replacement and evidence of functional efficacy in rodent models of retinal degeneration. Ongoing work has been directed toward the replication of these results ... Full text Link to item Cite

Phenotypic stability of Pro347Leu rhodopsin transgenic pigs as indicated by photoreceptor cell degeneration.

Journal Article Transgenic Res · December 2011 Rhodopsin (Pro347Leu) transgenic pigs are recognized to be an excellent model for the human disease, retinitis pigmentosa. First published in 1997, the rhodopsin transgenic pigs have been maintained since that time at North Carolina State University by out ... Full text Link to item Cite

Identification of variants in CNGA3 as cause for achromatopsia by exome sequencing of a single patient.

Journal Article Arch Ophthalmol · September 2011 OBJECTIVE: To report disease-causing mutations in the cyclic nucleotide-gated channel α 3 gene (CNGA3) identified by exome sequencing and bioinformatics filtering in a single patient. METHODS: The entire protein-coding sequence of a patient with a retinal ... Full text Link to item Cite

TLS interaction with NMDA R1 splice variant in retinal ganglion cell line RGC-5.

Journal Article Neurosci Lett · January 30, 2009 Translocated in liposarcoma (TLS or FUS) is a multifunctional protein component of the heterogenous ribonuclear complex involved in the splicing of pre-mRNA and the export of fully processed mRNA from the nucleus to the cytoplasm. As we determined that TLS ... Full text Link to item Cite

Porcine global flash multifocal electroretinogram: possible mechanisms for the glaucomatous changes in contrast response function.

Journal Article Vision Res · July 2008 PURPOSE: The aim of this study was to obtain a better understanding of the cellular contributions to the porcine global flash mfERG by using a pharmacologic dissection method, together with the method using variation of stimulus contrast which has been use ... Full text Link to item Cite

Pharmacologically defined components of the normal porcine multifocal ERG.

Journal Article Doc Ophthalmol · May 2008 Multifocal electroretinograms (mfERG) from isoflurane anesthetized pigs were recorded and sequential application of TTX, NMDA, APB and PDA were used to identify contributions to the mfERG from inner retinal neurons, ON-pathway, OFF-pathway and photorecepto ... Full text Link to item Cite

Multifocal electroretinogram in rhodopsin P347L transgenic pigs.

Journal Article Invest Ophthalmol Vis Sci · May 2008 PURPOSE: Neural ectopic rewiring in retinal degeneration such as retinitis pigmentosa (RP) may form functional synapses between cones and rod bipolar cells that cause atypical signal processing. In this study, the multifocal electroretinograms (mfERGs) of ... Full text Link to item Cite

Effect of cellular senescence on the P2Y-receptor mediated calcium response in trabecular meshwork cells.

Journal Article Mol Vis · October 16, 2007 PURPOSE: The objectives of this study were to evaluate the potential release of ATP that is mediated by mechanical stress on trabecular meshwork (TM) cells, to identify the specific P2Y receptors mediating the ATP response, and to determine whether cellula ... Link to item Cite

Targeted inactivation of synaptic HRG4 (UNC119) causes dysfunction in the distal photoreceptor and slow retinal degeneration, revealing a new function.

Journal Article Exp Eye Res · March 2007 HRG4 (UNC119) is a photoreceptor protein predominantly localized to the photoreceptor synapses and to the inner segments to a lesser degree. A heterozygous truncation mutation in HRG4 was found in a patient with late onset cone-rod dystrophy, and a transge ... Full text Link to item Cite

Altered light responses of single rod photoreceptors in transgenic pigs expressing P347L or P347S rhodopsin.

Journal Article Mol Vis · December 31, 2005 PURPOSE: Numerous mutations of rhodopsin lead to rod cell death and ultimately to complete blindness, yet little is known about the alterations in the physiology of the light sensors containing the aberrant protein, the rod photoreceptors. METHODS: Suction ... Link to item Cite

Oxidative damage is a potential cause of cone cell death in retinitis pigmentosa.

Journal Article J Cell Physiol · June 2005 Retinitis pigmentosa (RP) is a prevalent cause of blindness caused by a large number of different mutations in many different genes. The mutations result in rod photoreceptor cell death, but it is unknown why cones die. In this study, we tested the hypothe ... Full text Link to item Cite

Effects of indocyanine green on the retina and retinal pigment epithelium in a porcine model of retinal hole.

Journal Article Retina · April 2004 PURPOSE: This study was designed to emulate human macular hole surgery and to test the effects of indocyanine green (ICG) on the retina and retinal pigment epithelium (RPE). METHODS: Yorkshire Cross pigs (n = 23) underwent vitrectomy, separation of the pos ... Full text Link to item Cite

Loss of invaginating rod bipolar cell dendrites in degenerating rod terminals

Conference INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · April 1, 2004 Link to item Cite

Transplantation of full-thickness retina in the rhodopsin transgenic pig.

Journal Article Retina · February 2004 PURPOSE: To establish the morphology of full-thickness neuroretinal grafts transplanted to hosts with degenerative photoreceptor disease. METHODS: Twenty rhodopsin transgenic pigs received a neuroretinal sheet from a neonatal normal pig in one eye. Followi ... Full text Link to item Cite

Effects of indocyanine green dye (ICG) on the retina and retinal pigment epithelium in a porcine model of retinal hole

Conference INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · May 1, 2003 Link to item Cite

Lensectomy and vitrectomy decrease the rate of photoreceptor loss in rhodopsin P347L transgenic pigs.

Journal Article Graefes Arch Clin Exp Ophthalmol · April 2003 BACKGROUND: Photoreceptor degeneration in retinitis pigmentosa (RP) runs an inevitable, gradually progressive course. A wide variety of growth factors of different origins have been shown to slow the rate of degeneration in some rodent models of RP. Recent ... Full text Link to item Cite

Ectopic synaptogenesis during retinal degeneration in the royal college of surgeons rat.

Journal Article Neuroscience · 2003 Featured Publication Rod photoreceptor-specific mutations cause ectopic synapses to form between cone photoreceptor terminals and rod bipolar cell dendrites in degenerating retinas of rhodopsin transgenic (P347L) pigs and retinal degeneration mice. Since the mutations occur in ... Full text Link to item Cite

RGD peptide-assisted vitrectomy to facilitate induction of a posterior vitreous detachment: a new principle in pharmacological vitreolysis.

Journal Article Curr Eye Res · December 2002 PURPOSE: To evaluate a new concept in pharmacological vitreolysis by studying the efficacy of intravitreal RGD peptide-assisted vitrectomy in facilitating the separation of the posterior cortical vitreous from the retinal surface in an animal model. METHOD ... Full text Link to item Cite

Transplantation of full-thickness retina in the rhodopsin transgenic pig

Conference INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · May 1, 2002 Link to item Cite

Light-induced changes of PKC alpha immunoreactivity in rod bipolar cells of normal and RD mice

Conference INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · May 1, 2002 Link to item Cite

Conservation and regeneration of transgenic lines of swine by semen cryopreservation and artificial insemination.

Journal Article Lab Anim (NY) · January 2002 Full text Link to item Cite

Intravitreal injection of Arg-Gly-Asp (RGD) peptide facilitates surgical creation of a posterior vitreous detachment in young pigs

Conference INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · March 15, 2001 Link to item Cite

Ectopic synaptogenesis is a common step in the progression of hereditary retinal degenerations

Journal Article INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · March 15, 2001 Link to item Cite

An allele-specific hammerhead ribozyme gene therapy for a porcine model of autosomal dominant retinitis pigmentosa.

Journal Article Mol Vis · January 26, 2001 PURPOSE: To develop a hammerhead ribozyme-based gene therapy for a porcine model of autosomal dominant retinitis pigmentosa (ADRP). METHODS: Hammerhead ribozymes were developed and assayed in vitro against RNA targets homologous to the opsin P347S mutants ... Link to item Cite

Dispase facilitates posterior vitreous detachment during vitrectomy in young pigs.

Journal Article Retina · 2001 PURPOSE: To evaluate the role of intravitreal dispase in conjunction with pars plana vitrectomy to facilitate the creation of a posterior vitreous detachment (PVD) in young pig eyes. METHODS: Twenty-four eyes of 24 animals were randomized to receive an int ... Full text Link to item Cite

Early loss of synaptic protein PSD-95 from rod terminals of rhodopsin P347L transgenic porcine retina.

Journal Article Brain Res · December 1, 2000 Retinitis pigmentosa (RP), a type of retinal degeneration involving first rod and then slow cone photoreceptor degeneration, can be caused by any of a number of mutations in different genes. In the cases of mutations affecting rod-specific genes such as rh ... Full text Link to item Cite

Ectopic synaptogenesis in the mammalian retina caused by rod photoreceptor-specific mutations.

Journal Article Nat Neurosci · November 2000 In addition to rod photoreceptor loss, many mutations in rod photoreceptor-specific genes cause degeneration of other neuronal types. Identifying mechanisms of cell-cell interactions initiated by rod-specific mutations and affecting other retinal cells is ... Full text Link to item Cite

Dispase-assisted vitrectomy facilitates posterior vitreous detachment in young porcines

Conference INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · March 15, 2000 Link to item Cite

Synaptic plasticity in mammalian retinas induced by rod-specific mutations

Conference INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · March 15, 2000 Link to item Cite

Clear lens extraction decreases the rate of photoreceptor loss in rhodopsin P347L transgenic pigs

Conference INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · March 15, 2000 Link to item Cite

Increase of GABA content and GAD and GABA-T activities in rhodopsin P347L transgenic porcine retinas

Journal Article INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · March 15, 2000 Link to item Cite

Optical coherence tomography (OCT) abnormalities in rhodopsin mutant transgenic swine with retinal degeneration.

Journal Article Exp Eye Res · February 2000 Full text Link to item Cite

Cell death in age-related macular degeneration.

Journal Article Mol Vis · November 3, 1999 The cellular and molecular mechanisms underlying the death of photoreceptors and other retinal cells in age-related macular degeneration (AMD) remain poorly understood. Some of the questions for which answers need to be sought, and which are explicitly or ... Link to item Cite

Rhodopsin mutation induces ectopic cone-rod bipolar cell synaptic connections in transgenic swine.

Conference MOLECULAR BIOLOGY OF THE CELL · November 1, 1999 Link to item Cite

Neural retinal cell transplantation.

Journal Article Ophthalmology · October 1999 Full text Link to item Cite

Retinal rod photoreceptor-specific gene mutation perturbs cone pathway development.

Journal Article Neuron · July 1999 Rod-specific photoreceptor dystrophies are complicated by the delayed death of genetically normal neighboring cones. In transgenic (Tg) swine with a rod-specific (rhodopsin) gene mutation, cone photoreceptor physiology was normal for months but later decli ... Full text Link to item Cite

Rod and cone electroretinograms of young rhodopsin P347L transgenic pigs

Conference INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE · March 15, 1999 Link to item Cite

The cloning of GRK7, a candidate cone opsin kinase, from cone- and rod-dominant mammalian retinas.

Journal Article Mol Vis · December 8, 1998 PURPOSE: Desensitization in the rod cell of the mammalian retina is initiated when light-activated rhodopsin is phosphorylated by the G protein-coupled receptor kinase (GRK), GRK1, often referred to as rhodopsin kinase. A distinct kinase that specifically ... Link to item Cite

Rhodopsin transgenic pigs as a model for human retinitis pigmentosa.

Journal Article Invest Ophthalmol Vis Sci · April 1998 PURPOSE: To further characterize the retinas of Pro3471Leu rhodopsin transgenic pigs, a model for human retinitis pigmentosa. METHODS: Retinas from normal and transgenic pigs, newborn to 20 months old, were processed for light and electron microscopic immu ... Link to item Cite

Panretinal laser photocoagulation failed to retard degeneration in rhodopsin P347L transgentc pigs

Journal Article Investigative Ophthalmology and Visual Science · December 1, 1997 Purpose Various tines of evidence suggest that injury to the eye, or to the retina, may retard retina! degeneration in animal models, probably by causing retinal cells to release factors that may promote cell survival. In the RCS rat, preservation of retin ... Cite

Eye size and refraction in pigs with retinal degeneration

Journal Article Investigative Ophthalmology and Visual Science · December 1, 1997 Purpose. To assess refraction and post natal eye growth in pigs with early severe retinal degeneration. Methods. At age ïmonths, five Pro347Leu rhodopsin transgenic pigs that had early severe retinal degeneration and five controls were examined under gener ... Cite

Genetically engineered large animal model for studying cone photoreceptor survival and degeneration in retinitis pigmentosa.

Journal Article Nat Biotechnol · October 1997 Patients with retinitis pigmentosa (RP) typically develop night blindness early in life due to loss of rod photoreceptors. The remaining cone photoreceptors are the mainstay of their vision; however, over years or decades, these cones slowly degenerate, le ... Full text Link to item Cite