Breathing patterns in a mouse model with human-like Duchenne muscular dystrophy

At 6 months old, hDMDΔ52;mdx mice exhibit reduced maximal respiration and worsening respiratory pathology compared to 12 months.

• Duchenne muscular dystrophy is linked to a lack of dystrophin, leading to muscle inflammation and atrophy.
• Boys with Duchenne muscular dystrophy typically experience respiratory failure by their 2nd or 3rd decade of life.
• The hDMDΔ52;mdx mouse model carries a human DMD gene sequence with an exon 52 deletion, providing a more relevant evaluation for genetic medicines.
• Characterization revealed neuromuscular junction pathology and fibrosis in the diaphragm of hDMDΔ52;mdx mice.
• The findings suggest this mouse model may be useful for studying the effects of genetic therapies on respiratory function.

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