Slight alteration of the electroretinogram in mice lacking dystrophin dp71

David Cia1, Manuel Simonutti, Patrice E Fort

  • 1UMR Inserm 1107, Laboratoire de Biophysique Neurosensorielle, UFR Pharmacie, Clermont Université, Clermont-Ferrand, France.

Ophthalmic Research
|March 26, 2014
PubMed
Abstract

Insights

The absence of dystrophin 71 (Dp71) alone causes a minor reduction in electroretinogram (ERG) b-wave amplitude. Both Dp71 and Dp260 are necessary for normal ERG b-wave generation in mice.

Area of Science:

  • Neuroscience
  • Ophthalmology
  • Genetics

Background:

  • Duchenne muscular dystrophy (DMD) patients and mdx(Cv3) mice lacking both dystrophins Dp260 and Dp71 exhibit reduced electroretinogram (ERG) b-wave amplitude.
  • Mice lacking only Dp260 show normal ERG b-wave amplitudes, suggesting a role for Dp71.

Purpose of the Study:

  • To investigate the specific effect of dystrophin 71 (Dp71) absence on the electroretinogram (ERG).

Main Methods:

  • Ganzfeld electroretinograms (ERGs) were performed on dark-adapted Dp71-null mice and their littermates.
  • Scotopic and photopic flash ERGs were recorded across various light intensities.
  • Oscillatory potentials (OPs) were extracted from scotopic ERGs.

Main Results:

  • Dp71-null mice displayed a slight but significant reduction in scotopic ERG b-wave amplitudes.
  • Normal a-wave amplitudes and implicit times were observed in scotopic ERGs.
  • No significant changes in OPs or photopic ERGs were detected.

Conclusions:

  • The sole absence of Dp71 leads to a mild attenuation of the ERG b-wave amplitude.
  • Both dystrophin 71 (Dp71) and dystrophin 260 (Dp260) are collectively essential for normal ERG b-wave generation in mice.

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