A randomized, placebo-controlled trial of the benzoquinone idebenone in a mouse model of OPA1-related dominant optic atrophy reveals a limited therapeutic effect on retinal ganglion cell dendropathy and visual function
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- 作者：T.G. Smitha ; S. Setoa ; P. Ganneb ; M. Votrubaa ; c ; VotrubaM@cardiff.ac.uk" class="auth_mail" title="E-mail the corresponding author
- 关键词：DiI ; 1 ; 1&prime ; -dioctadecyl-3 ; 3 ; 3&prime ; 3&prime ; -tetramethylindocarbocyanine perchlorate ; GLC ; ganglion cell layer ; INL ; inner nuclear layer ; IS ; inner segment of photoreceptive layer ; NQO1 ; NAD(P)H ; quinone oxidoreductase-1 ; ONL ; outer nuclear layer ; TBARS ; thiobarbituric acid reactive species
- 刊名：Neuroscience
- 出版年：2016
- 出版时间：5 April 2016
- 年：2016
- 卷：319
- 期：Complete
- 页码：92-106
- 全文大小：2672 K
文摘
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We conducted a trial of idebenone in an Opa1 mouse model of dominant optic atrophy.
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We report the expression profile of NQO1 in the brain, retina and liver.
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Mutant mice receiving treatment showed an early improvement in visual acuity.
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In aged mice, vision and retinal dendritic loss were not prevented by idebenone.
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Idebenone had a deleterious effect on wildtype retinal ganglion cells.