In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa

Proceedings of the National Academy of Sciences of the United States of America
Artur V CideciyanGustavo D Aguirre


Genetic and environmental factors modify the severity of human neurodegenerations. Retinal degenerations caused by rhodopsin gene mutations show severity differences within and between families and even within regions of the same eye. Environmental light is thought to contribute to this variation. In the naturally occurring dog model of the human disorder, we found that modest light levels, as used in routine clinical practice, dramatically accelerated the neurodegeneration. Dynamics of acute retinal injury (consisting of abnormal intraretinal light scattering) were visualized in vivo in real time with high-resolution optical imaging. Long term consequences included fast or slow retinal degeneration or repair of injury depending on the dose of light exposure. These experiments provide a platform to study mechanisms of neuronal injury, repair, compensation, and degeneration. The data also argue for a gene-specific clinical trial of light reduction in human rhodopsin disease.


Dec 30, 1977·Documenta Ophthalmologica. Advances in Ophthalmology·T LawwillG Currier
Dec 30, 1977·Documenta Ophthalmologica. Advances in Ophthalmology·B Kovács
Nov 8, 1990·The New England Journal of Medicine·T P DryjaE L Berson
Jan 1, 1986·Cell and Tissue Research·M MoriyaT P Williams
Mar 15, 1985·American Journal of Ophthalmology·E L BersonA H Hanson
Aug 1, 1970·American Journal of Ophthalmology·T Kuwabara
Apr 1, 1995·Investigative Ophthalmology & Visual Science·T G Gorgels, D van Norren
Jun 1, 1997·Experimental Eye Research·F HafeziC E Remé
Jun 17, 1998·Proceedings of the National Academy of Sciences of the United States of America·A V CideciyanS G Jacobson
May 27, 1999·Vision Research·E M BuschD van Norren
Oct 26, 1999·Progress in Retinal and Eye Research·J StoneJ Peér
Apr 25, 2002·Proceedings of the National Academy of Sciences of the United States of America·James W KijasGregory M Acland
May 4, 2002·Nature Cell Biology·Eitan Shaulian, Michael Karin
Sep 10, 2002·Nature Genetics·Wenshan HaoJanis Lem
Oct 2, 2002·Nature Genetics·Samuel G Jacobson, Roderick R McInnes
Jan 31, 2003·Investigative Ophthalmology & Visual Science·Daniel T OrganisciakBarbara Wiggert
Apr 18, 2003·Human Molecular Genetics·Samuel G JacobsonEdwin M Stone
Oct 7, 2003·Annual Review of Neuroscience·Laura R PacioneRoderick R McInnes
Mar 25, 2004·Lancet Neurology·Xiongwei ZhuMark A Smith
Jun 29, 2004·Nature Genetics·Thierry LéveillardJosé-Alain Sahel
Aug 24, 2004·Nature Reviews. Neuroscience·Susanna C Benn, Clifford J Woolf
Aug 25, 2004·Experimental Eye Research·Babak Jian SeyedahmadiThaddeus P Dryja
Sep 8, 2004·Molecular Cell·Cole M HaynesAntony A Cooper
Dec 22, 2004·Progress in Retinal and Eye Research·Andreas WenzelCharlotte E Remé

❮ Previous
Next ❯


May 14, 2008·Archives of Ophthalmology·András M KomáromyGustavo D Aguirre
Nov 9, 2011·Mammalian Genome : Official Journal of the International Mammalian Genome Society·Keiko MiyaderaGustavo D Aguirre
Dec 24, 2011·Mammalian Genome : Official Journal of the International Mammalian Genome Society·Keiko MiyaderaDavid R Sargan
Oct 31, 2006·Graefe's Archive for Clinical and Experimental Ophthalmology = Albrecht Von Graefes Archiv Für Klinische Und Experimentelle Ophthalmologie·Fredrik GhoshRobert M Petters
Jun 28, 2011·Documenta Ophthalmologica. Advances in Ophthalmology·Dingcai CaoMichael A Grassi
May 14, 2009·Molecular Therapy : the Journal of the American Society of Gene Therapy·Annagiusi GargiuloEnrico M Surace
Sep 26, 2007·Human Gene Therapy·Daniel M PaskowitzDouglas Vollrath
Jul 9, 2010·Genetics in Medicine : Official Journal of the American College of Medical Genetics·William J KimberlingRichard J H Smith
May 19, 2006·The British Journal of Ophthalmology·D M PaskowitzJ L Duncan
Jun 8, 2006·Annual Review of Biochemistry·Krzysztof Palczewski
Apr 27, 2007·Investigative Ophthalmology & Visual Science·Karina E GuziewiczGustavo D Aguirre
Apr 4, 2008·Investigative Ophthalmology & Visual Science·Tomas S AlemanSamuel G Jacobson
Apr 15, 2008·Investigative Ophthalmology & Visual Science·Jessica I W MorganDavid R Williams
Aug 25, 2009·BMC Ophthalmology·Drew ScolesWilliam H Merigan
Jun 25, 2008·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·Jelena OstojicDonald S Sakaguchi
May 31, 2014·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Barbara VonaThomas Haaf
Dec 7, 2006·Ophthalmic Genetics·Ewa BudzynskiPatsy M Nishina
Dec 15, 2012·International Journal of Pediatric Otorhinolaryngology·Hidekane YoshimuraShin-ichi Usami
Sep 2, 2015·Experimental Eye Research·Yu ChenAkiko Maeda
Feb 10, 2010·Ophthalmology·Martin A Mainster, Patricia L Turner
Nov 17, 2009·Journal of Molecular Biology·Mark P KrebsShalesh Kaushal
May 6, 2009·Current Opinion in Genetics & Development·Edwin M Stone
Mar 11, 2008·Progress in Retinal and Eye Research·Camiel J F BoonJan E E Keunen
Nov 1, 2005·Clinical & Experimental Optometry : Journal of the Australian Optometrical Association·Michael Kalloniatis, Erica L Fletcher
Jul 11, 2006·Experimental Eye Research·Camilla JozwickJonathan Stone
May 24, 2006·Progress in Retinal and Eye Research·Rogério A CostaMaciej Wojtkowski
Dec 20, 2005·Experimental Eye Research·Dana K VaughanSteven J Fliesler
Feb 24, 2006·American Journal of Ophthalmology·Amir Rattner, Jeremy Nathans
Sep 30, 2014·Ophthalmic Genetics·Maria L NapierColin E Willoughby
Mar 18, 2015·Journal of Cellular and Molecular Medicine·Imene JaadaneAlicia Torriglia
Nov 17, 2011·Progress in Retinal and Eye Research·Jennifer J HunterDavid R Williams
Feb 22, 2008·Vision Research·Camiel J F BoonThomas Theelen
Apr 21, 2009·Vision Research·Wolfgang Baehr, Jeanne M Frederick
Feb 12, 2015·Human Gene Therapy. Clinical Development·Simon M Petersen-Jones, András M Komáromy
Dec 31, 2014·Human Gene Therapy. Clinical Development·Simon Petersen-Jones, Andras M Komaromy

❮ Previous
Next ❯

Related Concepts

Trending Feeds


Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.


Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.


Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

The British Journal of Ophthalmology
D M PaskowitzJ L Duncan
Proceedings of the National Academy of Sciences of the United States of America
James W KijasGregory M Acland
Proceedings of the National Academy of Sciences of the United States of America
N L MataG H Travis
Investigative Ophthalmology & Visual Science
Daniel T OrganisciakBarbara Wiggert
© 2021 Meta ULC. All rights reserved