Slight Alteration of the Electroretinogram in Mice Lacking Dystrophin Dp71

Aim: Most Duchenne muscular dystrophy patients and the mdx Cv3 mouse strain, lacking expression of both dystrophins Dp260 and Dp71, show a high attenuation of the dark-adapted electroretinogram (ERG) b-wave amplitude, whereas mice lacking the expression of Dp260 show normal b-wave amplitude. Here, w...

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Veröffentlicht in:Ophthalmic research 2014-01, Vol.51 (4), p.196-203
Hauptverfasser: Cia, David, Simonutti, Manuel, Fort, Patrice E., Doly, Michel, Rendon, Alvaro
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Sprache:eng
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Zusammenfassung:Aim: Most Duchenne muscular dystrophy patients and the mdx Cv3 mouse strain, lacking expression of both dystrophins Dp260 and Dp71, show a high attenuation of the dark-adapted electroretinogram (ERG) b-wave amplitude, whereas mice lacking the expression of Dp260 show normal b-wave amplitude. Here, we completed our assessment of whether the sole absence of Dp71 affects the ERG. Methods: Ganzfeld ERGs were performed on dark-adapted Dp71-null mice and littermates. Scotopic flash ERGs were recorded at light intensities from 3.10− 5 to 1 cd.s/m 2 . Oscillatory potentials (OPs) were extracted at 1 cd.s/m 2 . Photopic flash ERGs were recorded at 10 cd.s/m 2 after light adaptation. Results: Dp71-null mice showed a slight but significant reduction in b-wave amplitudes, normal a-wave amplitudes and nonaffected implicit times of the scotopic ERGs. No changes were observed in the amplitudes and implicit times of the OPs and the photopic ERGs. Conclusions: Our results demonstrate that together both Dp71 and Dp260 are required for the generation of the ERG b-wave in mice.
ISSN:0030-3747
1423-0259
DOI:10.1159/000357272