Altered social behavior and ultrasonic communication in the dystrophin-deficient mdx mouse model of Duchenne muscular dystrophy

摘要: Background The Duchenne and Becker muscular dystrophies (DMD, BMD) show significant comorbid diagnosis for autism, the genomic sequences encoding proteins responsible these diseases, dystrophin associated proteins, have been proposed as new candidate risk loci autism. Dystrophin is expressed not only in muscles but also central inhibitory synapses cerebellum, hippocampus, amygdala, cerebral cortex, where it contributes to organization of autism-associated trans-synaptic neurexin-neuroligin complexes clustering synaptic gamma-aminobutyric acid (GABA)A receptors. While brain defects due loss are with deficits cognitive executive functions, communication skills social behavior, a subpopulation DMD patients meet criteria suggesting that mutations gene may confer vulnerability The mdx mouse model has emotional alterations, behavior abilities never studied this model.