Behavioral improvement in dystrophic <i>mdx23</i> mouse following repeated antisense oligonucleotides injections.

ABSTRACT: Duchenne muscular dystrophy (DMD) is a severe neuromuscular disorder caused by mutations in the DMD gene that disrupt the production of functional dystrophin proteins. Intellectual disability and neurobehavioral complications including autism spectrum disorder, attention-deficit disorders, and anxiety cumulatively occur in 33%-43% of the patients due to deficiency of multiple dystrophin isoforms produced in brain. Previous work also identified behavioral abnormalities in the mdx23 mouse model of DMD. In this work we mapped the expression of the different dystrophin isoforms in different areas of the mouse brain. Next, we determined the behavioral phenotypes that best differentiate mdx23 (lacking the Dp427 isoform) and wild-type mice. Finally, we investigated the respon

SUBMITTER: Aghaeipour A 

PROVIDER: S-EPMC12744854 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature