April 2010
Volume 51, Issue 13
Free
ARVO Annual Meeting Abstract  |   April 2010
Heterozygous B6;C3-Opa1Q285stop Mouse Model of Dominant Optic Atrophy Displays Subtle Neuromuscular Features With Age but No Increase in Severity on a B6;C3-Opa3L122p Background
Author Affiliations & Notes
  • E. Taylor
    School of Optometry and Vision Science, Cardiff University, Cardiff, United Kingdom
  • J. Davies
    School of Optometry and Vision Science, Cardiff University, Cardiff, United Kingdom
  • K. Powell
    School of Optometry and Vision Science, Cardiff University, Cardiff, United Kingdom
  • V. Davies
    School of Psychology, Cardiff Neuroscience Centre, Cardiff, United Kingdom
  • M. Votruba
    School of Optometry and Vision Science, Cardiff University, Cardiff, United Kingdom
    Cardiff Eye Unit, University Hospital Wales, Cardiff, United Kingdom
  • Footnotes
    Commercial Relationships  E. Taylor, None; J. Davies, None; K. Powell, None; V. Davies, None; M. Votruba, None.
  • Footnotes
    Support  MRC project grant G0700949
Investigative Ophthalmology & Visual Science April 2010, Vol.51, 1451. doi:
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      E. Taylor, J. Davies, K. Powell, V. Davies, M. Votruba; Heterozygous B6;C3-Opa1Q285stop Mouse Model of Dominant Optic Atrophy Displays Subtle Neuromuscular Features With Age but No Increase in Severity on a B6;C3-Opa3L122p Background. Invest. Ophthalmol. Vis. Sci. 2010;51(13):1451.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: : OPA1

Methods: : In addition to published husbandry and genotyping protocols, we carried out genotyping by allele-specific PCR for a potential confounding mitochondrial gene deletion, Nnt, (carried by all C57Bl/6). With selective breeding this deletion was removed from our line. Body weight, SHIRPA test, accelerating rotarod and OKN was carried out. Opa1 x Opa3 lines were crossed.

Results: : Mean weights of all Opa1 male and female mice over 6 months age showed a trend towards lighter weight in Opa1+/- mice (WT 32.74g, SD=6.0; Het 29.47g, SD=2.7 ; p> 0.05). By 20 months of age there was a statistically significant difference between WT and Hets (WT 40.6g, SD=3.1; Het 28.77g, SD=2.44; p< 0.05). 12 month Opa1+/- showed reduced mean performance on rotarod (WT 92s, SD=10; Het 70s, SD=20.9; p=0.05). Rotarod testing at 20 months was discontinued since grossly obese wild type mice were unable to perform the test and act as controls. Opa1 x Opa3 cross revealed no evidence on SHIRPA or OKN testing of increased severity of phenotype (G2).

Conclusions: : Opa1+/- mice are lighter than WT age and sex matched controls. Rotarod testing confirms a subtle neuromuscular defect in Opa1+/- mice in line with previous SHIRPA testing. Opa1+/- mice on a mutant Opa3+/- background showed no evidence of more severe phenotype.

Keywords: neuro-ophthalmology: optic nerve • genetics • gene/expression 
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