論文種別	原著(症例報告除く)
言語種別	英語
査読の有無	その他(不明)
表題	Electrophysiological Evaluation in mdx52 Mouse Brain After Antisense-Mediated Exon 51 or 53 Skipping.
掲載誌名	正式名：Methods in molecular biology (Clifton, N.J.) 略　 称：Methods Mol Biol ISSNコード：19406029/10643745
掲載区分	国外
巻・号・頁	2964,pp.277-282
著者・共著者	Yasumasa Hashimoto, Yoshitsugu Aoki
発行年月	2025
概要	Duchenne muscular dystrophy (DMD) is a neuromuscular disease characterized by progressive muscle weakness alongside cognitive and behavioral impairments. Notably, the lack of brain-specific Dp140 isoform increases the risk of neurodevelopmental disorders in DMD. We reported abnormal social behavior and the reduced glutamatergic transmission using electrophysiological techniques in DMD model mice lacking Dp140. Also, we proposed that antisense oligonucleotide (ASO) -induced exon 53 skipping therapy is thought to be promising for neurodevelopmental disorders in DMD lacking Dp140. Here, we describe a detailed protocol for the intracerebroventricular injection and electrophysiological evaluation in the brain of DMD model mice following ASO-induced exon 51 or 53 skipping therapy.
DOI	10.1007/978-1-0716-4730-1_18
PMID	40720025