Expression in the mammalian retina of parkin and UCH-L1, two components of the ubiquitin-proteasome system

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Title: Expression in the mammalian retina of parkin and UCH-L1, two components of the ubiquitin-proteasome system
Authors: Esteve Rudd, Julián | Campello Blasco, Laura | Herrero Ezquerro, María Trinidad | Cuenca, Nicolás | Martín-Nieto, José
Research Group/s: Neurobiología del Sistema Visual y Terapia de Enfermedades Neurodegenerativas (NEUROVIS) | Genética Humana y de Mamíferos (GHM)
Center, Department or Service: Universidad de Alicante. Departamento de Fisiología, Genética y Microbiología
Keywords: Retina | Proteasome | Parkin | UCH-L1 | Gene expression | Parkinson disease
Knowledge Area: Oftalmología | Fisiología
Issue Date: 15-Jul-2010
Publisher: Elsevier
Citation: ESTEVE RUDD, Julián, et al. "Expression in the mammalian retina of parkin and UCH-L1, two components of the ubiquitin-proteasome system". Brain Research. Vol. 1352 (17 Sept. 2010). ISSN 0006-8993, pp. 70-82
Abstract: The ubiquitin-proteasome system (UPS) functions as a major degradation pathway for misfolded and damaged proteins with an important neuroprotective role in the CNS against a variety of cellular stresses. Parkin and ubiquitin C-terminal hydrolase L1 (UCH-L1) are two relevant components of the UPS associated with a number of neurodegenerative disorders. We here address the expression profile of parkin and UCH-L1 in the mammalian retina, with special emphasis on primates. We describe for the first time the presence of parkin in the retina of mammals, including humans. Parkin and UCH-L1 genes were expressed at the mRNA and protein levels in the retina of all species examined. The immunolocalization pattern of parkin was quite widespread, being expressed by most retinal neuronal types, including photoreceptors. UCH-L1 was localized to horizontal cells and specific subtypes of bipolar and amacrine cells, as well as to ganglion cells and their axons forming the nerve fiber layer. In rodents no UCH-L1 immunoreactivity was found in cone or rod photoreceptors, whereas this protein was present along the whole length of cones in all other mammals. Remarkably, UCH-L1 was expressed by dopaminergic amacrine cells of primates. The ample distribution of parkin and UCH-L1 in the mammalian retina, together with the crucial role played by the UPS in normal neuronal physiology in the brain, points to a participation of these two proteins in the ubiquitin-proteasomal pathway of protein degradation in most retinal cell types, where they could exert a protective function against neuronal stress.
Sponsor: This research was supported by grants from the Spanish Instituto de Salud Carlos III (ISCIII; PI09/1623) to J.M.-N., Ministerio de Ciencia e Innovación (MICINN; SAF2007-62262), Fundación Séneca (FS/05662/PI/07) and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED) to M.-T.H.; and MICINN (BFU2009-07793/BFI), ISCIII (RETICS RD07/0062/0012), Generalitat Valenciana (ACOMP/2009/139), Organización Nacional de Ciegos de España (ONCE) and Fundación Lucha contra la Ceguera (FUNDALUCE) to N.C. J.E.-R. and L.C. are recipients of predoctoral contracts from the Universidad de Alicante.
ISSN: 0006-8993 (Print) | 1872-6240 (Online)
DOI: 10.1016/j.brainres.2010.07.019
Language: eng
Type: info:eu-repo/semantics/article
Peer Review: si
Publisher version:
Appears in Collections:INV - NEUROVIS - Artículos de Revistas
INV - GHM - Artículos de Revistas

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