Mitochondrial Protection by Exogenous Otx2 in Mouse Retinal Neurons

Autor: Serge Picaud, Manuel Simonutti, Thomas Lamonerie, In-Beom Kim, Minho Shong, Hyoung Tai Kim, Takahisa Furukawa, Young In Sohn, Kyeong Hwan Moon, Romain Caplette, Kwang Wook Min, Antonio Simeone, Jong Soon Choi, Sun Sook Paik, Mi Jeong Kim, Hee Seok Kweon, Jin Woo Kim, Dong-Gi Lee, Soung Jung Kim, Eun Jung Lee
Přispěvatelé: Department of Biological Sciences, Seoul National University [Seoul] (SNU), Chungnam National University (CNU), Catholic University of Korea, Institut de la Vision, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Nano-Bio Electron Microscopy Research Group, Biological Disaster Analysis Group, Institute of Genetics and Biophysics 'A. Buzzati Traverso', Consiglio Nazionale delle Ricerche [Roma] (CNR), Istituto Neurologico Mediterraneo (NEUROMED I.R.C.C.S.), Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome]-Università degli studi di Napoli Federico II, Institut de Biologie Valrose (IBV), Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Côte d'Azur (UCA)-Centre National de la Recherche Scientifique (CNRS), Institute for Protein Research [Osaka], Osaka University [Osaka], Japan Agency for Medical Research and Development (AMED), National Research Council of Italy | Consiglio Nazionale delle Ricerche (CNR), Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome] (UNIROMA)-University of Naples Federico II = Università degli studi di Napoli Federico II, Université Nice Sophia Antipolis (1965 - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Côte d'Azur (UCA)
Jazyk: angličtina
Rok vydání: 2015
Předmět:
Zdroj: Cell reports 13 (2015): 990–1002. doi:10.1016/j.celrep.2015.09.075
info:cnr-pdr/source/autori:Kim H.-T.; Kim S.J.; Sohn Y.-I.; Paik S.-S.; Caplette R.; Simonutti M.; Moon K.H.; Lee E.J.; Min K.W.; Kim M.J.; Lee D.-G.; Simeone A.; Lamonerie T.; Furukawa T.; Choi J.-S.; Kweon H.-S.; Picaud S.; Kim I.-B.; Shong M.; Kim J.W./titolo:Mitochondrial Protection by Exogenous Otx2 in Mouse Retinal Neurons/doi:10.1016%2Fj.celrep.2015.09.075/rivista:Cell reports/anno:2015/pagina_da:990/pagina_a:1002/intervallo_pagine:990–1002/volume:13
Cell Reports
Cell Reports, Elsevier Inc, 2015, 13 (5), pp.990-1002. ⟨10.1016/j.celrep.2015.09.075⟩
Cell Reports, 2015, 13 (5), pp.990-1002. ⟨10.1016/j.celrep.2015.09.075⟩
CELL REPORTS(13): 5
Cell Reports, Vol 13, Iss 5, Pp 990-1002 (2015)
ISSN: 2211-1247
Popis: International audience; OTX2 (orthodenticle homeobox 2) haplodeficiency causes diverse defects in mammalian visual systems ranging from retinal dysfunction to anophthalmia. We find that the retinal dystrophy of Otx2+/GFP heterozygous knockin mice is mainly due to the loss of bipolar cells and consequent deficits in retinal activity. Among bipolar cell types, OFF-cone bipolar subsets, which lack autonomous Otx2 gene expression but receive Otx2 proteins from photoreceptors, degenerate most rapidly in Otx2+/GFP mouse retinas, suggesting a neuroprotective effect of the imported Otx2 protein. In support of this hypothesis, retinal dystrophy in Otx2+/GFP mice is prevented by intraocular injection of Otx2 protein, which localizes to the mitochondria of bipolar cells and facilitates ATP synthesis as a part of mitochondrial ATP synthase complex. Taken together, our findings demonstrate a mitochondrial function for Otx2 and suggest a potential therapeutic application of OTX2 protein delivery in human retinal dystrophy.
Databáze: OpenAIRE
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