Slight alteration of the electroretinogram in mice lacking dystrophin dp71

Ophthalmic Res. 2014;51(4):196-203. doi: 10.1159/000357272. Epub 2014 Mar 22.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Dark Adaptation
  • Disease Models, Animal
  • Dystrophin / physiology*
  • Electroretinography*
  • Mice
  • Mice, Inbred mdx
  • Muscular Dystrophy, Animal / physiopathology*
  • Photic Stimulation
  • Retina / physiopathology*

Substances

  • Dystrophin
  • apo-dystrophin 1