Mitochondrial optic neuropathies – Disease mechanisms and therapeutic strategies
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TL;DR: Optic nerve degeneration in LHON and DOA is therefore due to disturbed mitochondrial function and a predominantly complex I respiratory chain defect has been identified using both in vitro and in vivo biochemical assays.
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About: This article is published in Progress in Retinal and Eye Research. The article was published on 01 Mar 2011. and is currently open access. The article focuses on the topics: Mitochondrial optic neuropathies & Leber's hereditary optic neuropathy.
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Citations
Efficacy and Safety of rAAV2-ND4 Treatment for Leber’s Hereditary Optic Neuropathy
Xing Wan,Han Pei,Min-jian Zhao,Shuo Yang,Weikun Hu,Heng He,Si-qi Ma,Ge Zhang,Xiao-yan Dong,Chen Chen,Dao Wen Wang,Bin Li +11 more
TL;DR: Findings support the feasible use of gene therapy for LHON by evaluating the efficacy and safety of a recombinant adeno-associated virus 2 carrying ND4 (rAAV2-ND4) in LHON patients carrying the G11778A mutation.
•Journal Article
Idebenone Treatment In Leber 's Hereditary Optic Neuropathy
Valerio Carelli,Chiara La Morgia,Maria Lucia Valentino,Michele Carbonelli,Alfredo A. Sadun,Divya Aggarwal,Rocco Liguori,Patrizia Avoni,Pasquale Montagna,Piero Barboni +9 more
TL;DR: To evaluate retrospectively the efficacy of idebenone therapy, the results presented by Klopstock et al. (2011) concerning the RHODOS study on a clinical trial with Idebenone in Leber's hereditary optic neuropathy (LHON) were reviewed.
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A neurodegenerative perspective on mitochondrial optic neuropathies.
Patrick Yu-Wai-Man,Patrick Yu-Wai-Man,Patrick Yu-Wai-Man,Marcela Votruba,Marcela Votruba,Florence Burté,Chiara La Morgia,Piero Barboni,Valerio Carelli +8 more
TL;DR: These so-called “plus” phenotypes are mechanistically important as they put the loss of RGCs within the broader perspective of neuronal loss and mitochondrial dysfunction, highlighting common pathways that could be modulated to halt progressive neurodegeneration in other related CNS disorders.
Oocytes maintain ROS-free mitochondrial metabolism by suppressing complex I
Aida Rodríguez-Nuevo,Ariadna Torres-Sanchez,Juan M. Duran,Cristian de Guirior,María Ángeles Martínez-Zamora,E. Böke +5 more
TL;DR: In this paper , the authors show that oocytes evade reactive oxygen species (ROS) by remodelling the mitochondrial electron transport chain through elimination of complex I. Complex I suppression represents an evolutionarily conserved strategy that allows longevity while maintaining biological activity in long-lived oocytes.
Mitochondrial dynamics, transport, and quality control: A bottleneck for retinal ganglion cell viability in optic neuropathies.
Yoko Ito,Adriana Di Polo +1 more
TL;DR: The present review focuses on the progress made to date on understanding the biology of mitochondrial dynamics and quality control and how dysregulation of these processes can profoundly affect retinal ganglion cell viability and function in optic nerve diseases.
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