The ubiquitin-proteasome system in retinal health and disease
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Title: | The ubiquitin-proteasome system in retinal health and disease |
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Authors: | Campello Blasco, Laura | Esteve Rudd, Julián | Cuenca, Nicolás | Martín-Nieto, José |
Research Group/s: | Genética Humana y de Mamíferos (GHM) | Neurobiología del Sistema Visual y Terapia de Enfermedades Neurodegenerativas (NEUROVIS) |
Center, Department or Service: | Universidad de Alicante. Departamento de Fisiología, Genética y Microbiología |
Keywords: | Neurodegenerative disorders | Oxidative stress | Proteasome | Retinal diseases | Ubiquitin |
Knowledge Area: | Biología Celular | Genética |
Issue Date: | 22-Jan-2013 |
Publisher: | Springer Science+Business Media |
Citation: | CAMPELLO, Laura, et al. “The ubiquitin-proteasome system in retinal health and disease”. Molecular Neurobiology. Vol. 47, Issue 2 (Apr. 2013). ISSN 0893-7648, pp. 790-810 |
Abstract: | The ubiquitin–proteasome system (UPS) is the main intracellular pathway for modulated protein turnover, playing an important role in the maintenance of cellular homeostasis. It also exerts a protein quality control through degradation of oxidized, mutant, denatured, or misfolded proteins and is involved in many biological processes where protein level regulation is necessary. This system allows the cell to modulate its protein expression pattern in response to changing physiological conditions and provides a critical protective role in health and disease. Impairments of UPS function in the central nervous system (CNS) underlie an increasing number of genetic and idiopathic diseases, many of which affect the retina. Current knowledge on the UPS composition and function in this tissue, however, is scarce and dispersed. This review focuses on UPS elements reported in the retina, including ubiquitinating and deubiquitinating enzymes (DUBs), and alternative proteasome assemblies. Known and inferred roles of protein ubiquitination, and of the related, SUMO conjugation (SUMOylation) process, in normal retinal development and adult homeostasis are addressed, including modulation of the visual cycle and response to retinal stress and injury. Additionally, the relationship between UPS dysfunction and human neurodegenerative disorders affecting the retina, including Alzheimer's, Parkinson's, and Huntington's diseases, are dealt with, together with numerous instances of retina-specific illnesses with UPS involvement, such as retinitis pigmentosa, macular degenerations, glaucoma, diabetic retinopathy (DR), and aging-related impairments. This information, though still basic and limited, constitutes a suitable framework to be expanded in incoming years and should prove orientative toward future therapy design targeting sight-affecting diseases with a UPS underlying basis. |
Sponsor: | Research at the authors' laboratory is supported by the Instituto de Salud Carlos III (grant ref. PI09/1623, to J.M.-N.). L.C. was the recipient of a predoctoral contract from the Universidad de Alicante. |
URI: | http://hdl.handle.net/10045/27935 |
ISSN: | 0893-7648 (Print) | A6632446 | 1559-1182 (Online) |
DOI: | 10.1007/s12035-012-8391-5 |
Language: | eng |
Type: | info:eu-repo/semantics/article |
Rights: | The original publication is available at www.springerlink.com |
Peer Review: | si |
Publisher version: | http://dx.doi.org/10.1007/s12035-012-8391-5 |
Appears in Collections: | INV - GHM - Artículos de Revistas INV - NEUROVIS - Artículos de Revistas |
Files in This Item:
File | Description | Size | Format | |
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Campello_2013_Mol_Neurobiol.pdf | Versión final (acceso restringido) | 615,96 kB | Adobe PDF | Open Request a copy |
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