Publications by authors named "Delphine Le Verger"
Article Synopsis
- Duchenne and Becker muscular dystrophies (DMD, BMD) are neuromuscular disorders linked to cognitive and behavioral issues, with studies suggesting that the severity of these issues may be connected to the loss of different dystrophin proteins.
- This study focused on Dp71-null mice, which lack the shortest dystrophin isoform, revealing abnormal social behaviors, vocalization, and changes in anxiety levels, but no impact on myopathy or learning/memory related to fear.
- The findings suggest that mutations affecting Dp71 might contribute to social and emotional problems commonly seen in DMD, supporting the idea that losing multiple dystrophin isoforms can exacerbate behavioral issues.
Recent emphasis has been placed on the role that cerebellar dysfunctions could have in the genesis of cognitive deficits in Duchenne muscular dystrophy (DMD). However, relevant genotype-phenotype analyses are missing to define whether cerebellar defects underlie the severe cases of intellectual deficiency that have been associated with genetic loss of the smallest product of the gene, the Dp71 dystrophin. To determine for the first time whether Dp71 loss could affect cerebellar physiology and functions, we have used patch-clamp electrophysiological recordings in acute cerebellar slices and a cerebellum-dependent behavioral test battery addressing cerebellum-dependent motor and non-motor functions in null transgenic mice.
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