Opa1 deficiency in a mouse model of dominant optic atrophy leads to retinal ganglion cell dendropathy

P. A. Williams; J. E. Morgan; M. Votruba

doi:10.1093/brain/awq218

Opa1 deficiency in a mouse model of dominant optic atrophy leads to retinal ganglion cell dendropathy

Brain ◽

10.1093/brain/awq218 ◽

2010 ◽

Vol 133 (10) ◽

pp. 2942-2951 ◽

Cited By ~ 60

Author(s):

P. A. Williams ◽

J. E. Morgan ◽

M. Votruba

Keyword(s):

Mouse Model ◽

Ganglion Cell ◽

Retinal Ganglion Cell ◽

Optic Atrophy ◽

Retinal Ganglion ◽

Dominant Optic Atrophy

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Electrophysiological and Histologic Assessment of Retinal Ganglion Cell Fate in a Mouse Model forOPA1-Associated Autosomal Dominant Optic Atrophy

Investigative Opthalmology & Visual Science ◽

10.1167/iovs.09-3606 ◽

2010 ◽

Vol 51 (3) ◽

pp. 1424 ◽

Cited By ~ 35

Author(s):

Peter Heiduschka ◽

Sven Schnichels ◽

Nico Fuhrmann ◽

Sabine Hofmeister ◽

Ulrich Schraermeyer ◽

...

Keyword(s):

Mouse Model ◽

Ganglion Cell ◽

Cell Fate ◽

Retinal Ganglion Cell ◽

Optic Atrophy ◽

Autosomal Dominant ◽

Retinal Ganglion ◽

Autosomal Dominant Optic Atrophy ◽

Dominant Optic Atrophy

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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

Neuroscience ◽

10.1016/j.neuroscience.2016.01.042 ◽

2016 ◽

Vol 319 ◽

pp. 92-106 ◽

Cited By ~ 13

Author(s):

T.G. Smith ◽

S. Seto ◽

P. Ganne ◽

M. Votruba

Keyword(s):

Mouse Model ◽

Ganglion Cell ◽

Retinal Ganglion Cell ◽

Therapeutic Effect ◽

Optic Atrophy ◽

Visual Function ◽

Controlled Trial ◽

Retinal Ganglion ◽

Dominant Optic Atrophy

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Red light provides partial protection against retinal ganglion cell degeneration in a mouse model of dominant optic atrophy through the activation of NFkB

Acta Ophthalmologica ◽

10.1111/j.1755-3768.2017.02672 ◽

2017 ◽

Vol 95 ◽

Author(s):

M. Votruba ◽

K. Beirne ◽

M. Rozanowska

Keyword(s):

Mouse Model ◽

Ganglion Cell ◽

Retinal Ganglion Cell ◽

Optic Atrophy ◽

Red Light ◽

Retinal Ganglion ◽

Cell Degeneration ◽

Partial Protection ◽

Dominant Optic Atrophy

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OPA1 gene therapy prevents retinal ganglion cell loss in a Dominant Optic Atrophy mouse model

Scientific Reports ◽

10.1038/s41598-018-20838-8 ◽

2018 ◽

Vol 8 (1) ◽

Cited By ~ 15

Author(s):

Emmanuelle Sarzi ◽

Marie Seveno ◽

Camille Piro-Mégy ◽

Lucie Elzière ◽

Mélanie Quilès ◽

...

Keyword(s):

Gene Therapy ◽

Mouse Model ◽

Ganglion Cell ◽

Retinal Ganglion Cell ◽

Optic Atrophy ◽

Cell Loss ◽

Retinal Ganglion ◽

Dominant Optic Atrophy ◽

Opa1 Gene

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Early Macular Retinal Ganglion Cell Loss in Dominant Optic Atrophy: Genotype-Phenotype Correlation

American Journal of Ophthalmology ◽

10.1016/j.ajo.2014.05.034 ◽

2014 ◽

Vol 158 (3) ◽

pp. 628-636.e3 ◽

Cited By ~ 29

Author(s):

Piero Barboni ◽

Giacomo Savini ◽

Maria Lucia Cascavilla ◽

Leonardo Caporali ◽

Jacopo Milesi ◽

...

Keyword(s):

Ganglion Cell ◽

Retinal Ganglion Cell ◽

Optic Atrophy ◽

Cell Loss ◽

Retinal Ganglion ◽

Phenotype Correlation ◽

Dominant Optic Atrophy ◽

Genotype Phenotype Correlation

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Pattern of retinal ganglion cell loss in dominant optic atrophy due to OPA1 mutations

Eye ◽

10.1038/eye.2011.2 ◽

2011 ◽

Vol 25 (5) ◽

pp. 596-602 ◽

Cited By ~ 34

Author(s):

P Yu-Wai-Man ◽

M Bailie ◽

A Atawan ◽

P F Chinnery ◽

P G Griffiths

Keyword(s):

Ganglion Cell ◽

Retinal Ganglion Cell ◽

Optic Atrophy ◽

Cell Loss ◽

Retinal Ganglion ◽

Dominant Optic Atrophy

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The Pattern of Retinal Ganglion Cell Loss in OPA1-Related Autosomal Dominant Optic Atrophy Inferred From Temporal, Spatial, and Chromatic Sensitivity Losses

Investigative Opthalmology & Visual Science ◽

10.1167/iovs.16-20309 ◽

2017 ◽

Vol 58 (1) ◽

pp. 502 ◽

Cited By ~ 4

Author(s):

Anna Majander ◽

Catarina João ◽

Andrew T. Rider ◽

G. Bruce Henning ◽

Marcela Votruba ◽

...

Keyword(s):

Ganglion Cell ◽

Retinal Ganglion Cell ◽

Optic Atrophy ◽

Autosomal Dominant ◽

Cell Loss ◽

Retinal Ganglion ◽

Autosomal Dominant Optic Atrophy ◽

Dominant Optic Atrophy ◽

Chromatic Sensitivity

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XIAP gene therapy effects on retinal ganglion cell structure and function in a mouse model of glaucoma

Gene Therapy ◽

10.1038/s41434-021-00281-7 ◽

2021 ◽

Author(s):

Shagana Visuvanathan ◽

Adam N. Baker ◽

Pamela S. Lagali ◽

Stuart G. Coupland ◽

Garfield Miller ◽

...

Keyword(s):

Gene Therapy ◽

Mouse Model ◽

Ganglion Cell ◽

Retinal Ganglion Cell ◽

Cell Structure ◽

Structure And Function ◽

Retinal Ganglion ◽

And Function

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Developmental Expression Profile of the Optic Atrophy Gene Product: OPA1 Is Not Localized Exclusively in the Mammalian Retinal Ganglion Cell Layer

Investigative Opthalmology & Visual Science ◽

10.1167/iovs.03-1093 ◽

2004 ◽

Vol 45 (6) ◽

pp. 1667 ◽

Cited By ~ 33

Author(s):

Saima Aijaz ◽

Lynda Erskine ◽

Glen Jeffery ◽

Shomi S. Bhattacharya ◽

Marcela Votruba

Keyword(s):

Ganglion Cell ◽

Gene Product ◽

Retinal Ganglion Cell ◽

Expression Profile ◽

Optic Atrophy ◽

Ganglion Cell Layer ◽

Cell Layer ◽

Developmental Expression ◽

Retinal Ganglion ◽

Retinal Ganglion Cell Layer

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Retina-specific loss ofIkbkap/Elp1causes mitochondrial dysfunction that leads to selective retinal ganglion cell degeneration in a mouse model of familial dysautonomia

Disease Models & Mechanisms ◽

10.1242/dmm.033746 ◽

2018 ◽

Vol 11 (7) ◽

pp. dmm033746 ◽

Cited By ~ 2

Author(s):

Yumi Ueki ◽

Veronika Shchepetkina ◽

Frances Lefcort

Keyword(s):

Mouse Model ◽

Mitochondrial Dysfunction ◽

Ganglion Cell ◽

Retinal Ganglion Cell ◽

Familial Dysautonomia ◽

Retinal Ganglion ◽

Cell Degeneration ◽

Specific Loss

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