Definition
The vertebrate retina is susceptible to a number of inherited retinal conditions (Inherited retinal conditions or inherited retinal diseases) that lead to vision loss. Although relatively few people inherit these devastating diseases (1:4000), they have been the target of considerable research effort. This is largely because many of the events that occur in retinal degeneration are also features of more common retinal conditions such as Age-related macular degeneration (AMD); treatments developed that prevent or slow retinal degeneration are likely to have broad application in the management of retinal disease. In addition, the mechanisms that lead to photoreceptor (Photoreceptors) death are common to neuronal death in other regions of the Central Nervous System (CNS). The retina is an easily accessible part of the CNS and therefore much can be gained about the function of neurons in general by examining specific aspects of retinal function. The purpose of this chapter is...
This is a preview of subscription content, log in via an institution to check access.
References
Wässle H (2004) Parallel processing in the mammalian retina. Nat Rev Neurosci 5:747–757
Marc RE, Jones BW, Watt CB, Strettoi E (2003) Neural remodeling in retinal degeneration. Prog Retin Eye Res 22:607–655
Marc RE, Jones BW, Anderson JR, Kinard K, Marshak DW, Wilson JH, Wensel T, Lucas RJ (2007) Neural reprogramming in retinal degeneration. Invest Ophthalmol Vis Sci. 48:3364–3371
Strettoi E, Pignatelli V (2000) Modifications of retinal neurons in a mouse model of retinitis pigmentosa. Proc Natl Acad Sci USA 97:11020–11025
Sun D, Bui BV, Vingrys AJ, Kalloniatis AJ (2007) Alterations in photoreceptor-bipolar cell signaling following ischemia/reperfusion in the rat retina. J Comp Neurol 505:131–146
Kalloniatis M, Fletcher EL (2004) Retinitis pigmentosa: understanding the clinical presentation, mechanisms and treatment options. Clin Exp Optom 87:65–80
Hartong DT, Berson EL, Dryja TP (2006) Retinitis pigmentosa. Lancet 368:1795–1809
Cideciyan AV, Jacobson SG, Aleman TS, Gu D, Pearce-Kelling SE, Sumaroka A, Acland GM, Aguirre GD (2005) In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa. Proc Natl Acad Sci USA 102:5233–5238
Röhrer B, Blanco R, Marc RE, Lloyd MB, Bok D, Schneeweis DM, Reichardt LF (2004) Functionally intact glutamate-mediated signaling in bipolar cells of the TRKB knockout mouse retina. Vis Neurosci 21:703–713
Yu DY, Cringle S, Valter K, Walsh N, Lee D, Stone J (2004) Photoreceptor death, trophic factor expression, retinal oxygen status, and photoreceptor function in the P23H rat. Invest Ophthalmol Vis Sci 45:2013–2019
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2009 Springer-Verlag GmbH Berlin Heidelberg
About this entry
Cite this entry
Fletcher, E.L., Acosta, M.L., Kalloniatis, M. (2009). Inherited Retinal Degenerations. In: Binder, M.D., Hirokawa, N., Windhorst, U. (eds) Encyclopedia of Neuroscience. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-29678-2_2446
Download citation
DOI: https://doi.org/10.1007/978-3-540-29678-2_2446
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-23735-8
Online ISBN: 978-3-540-29678-2
eBook Packages: Biomedical and Life SciencesReference Module Biomedical and Life Sciences