Progressive Optic Atrophy in a Retinal Ganglion Cell-Specific Mouse Model of Complex I Deficiency (Video)

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Identifier 20200310_nanos_sciplatform3_03
Title Progressive Optic Atrophy in a Retinal Ganglion Cell-Specific Mouse Model of Complex I Deficiency (Video)
Creator Sidney M. Gospe, III, Luyu Wang, Mikael Klingeborn, Amanda Travis, Ying Hao, Vadim Arshavsky
Subject Optic Neuropathy, Genetic Disease
Description Optic atrophy resulting from retinal ganglion cell (RGC) degeneration is the most prominent ocular manifestation of mitochondrial dysfunction. Efforts to develop effective pharmacotherapies for mitochondrial optic neuropathies have been hampered, in part, by the lack of an ideal preclinical animal model. Although mutant mice lacking the mitochondrial complex I accessory subunit NADH:ubiquinone oxidoreductase (NDUFS4) develop early-onset optic atrophy, severe systemic mitochondrial dysfunction leads to very early death, making this mouse line impractical for studying the pathobiology of mitochondrial optic neuropathy.
Date 2020-03
Language eng
Format video/mp4
Source 2020 North American Neuro-Ophthalmology Society Annual Meeting
Relation is Part of NANOS Annual Meeting 2020: Scientific Platform Session III
Collection Neuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Holding Institution North American Neuro-Ophthalmology Association. NANOS Executive Office 5841 Cedar Lake Road, Suite 204, Minneapolis, MN 55416
Rights Management Copyright 2020. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s6vx5t29
Context URL The NANOS Annual Meeting Neuro-Ophthalmology Collection: https://novel.utah.edu/collection/NAM/toc/
Contributor Primary Sidney M. Gospe, III, MD, PhD
Contributor Secondary Luyu Wang, Mikael Klingeborn, Amanda Travis, Ying Hao, Vadim Arshavsky
Setname ehsl_novel_nam
ID 1544739
Reference URL https://collections.lib.utah.edu/ark:/87278/s6vx5t29