Tomonori Kitaura, Translational Research Business Unit, will present a poster at the 31st annual Congress of the World Muscle Society

2026.09.28

Tomonori Kitaura of the Translational Research Business Unit will deliver a poster presentation at 31st Annual Congress of the World Muscle Society,  to be held at International Congress Center Hiroshima (ICCH), Hiroshima prefecture, Japan.
If you plan to attend the conference, we encourage you to join the session and hear the presentation.

Conference Name: 31st Annual Congress of the World Muscle Society

Date & Time: September 30, 2026 (17:15-18:15 JST)

Location: International Congress Center Hiroshima (ICCH), Hiroshima prefecture, Japan

Title: Early cardiac dysfunction in mdx52/Utrn^-/- mice as a severe preclinical model for Duchenne muscular dystrophy

No: 2.72eP

Presentation Summary
Duchenne muscular dystrophy (DMD) is a severe disease characterized by progressive skeletal muscle degeneration accompanied by cardiomyopathy and respiratory dysfunction. Although B10-mdx mice are widely used in DMD research, their relatively mild phenotype and delayed disease onset compared with human DMD present challenges for drug discovery studies.
At Axcelead, we generated mdx52/Utrn-/- mice lacking dystrophin and utrophin and characterized their disease progression over time. While diastolic dysfunction has been reported in B10-mdx mice at around 12 months of age, mdx52//Utrn-/- mice exhibited deterioration in multiple echocardiographic parameters indicative of diastolic function as early as 9 weeks of age.
. In addition, skeletal muscle dysfunction was observed in grip strength, treadmill, and rotarod tests. These findings suggest that this model can capture both cardiac and skeletal muscle dysfunction observed in patients with DMD from an early stage and is expected to be a valuable preclinical evaluation model for assessing therapeutic efficacy.

Axcelead’s Soulution
Axcelead provides a preclinical evaluation platform that combines highly translational disease models with comprehensive functional assessment technologies.
For DMD research, in addition to the conventional B10-mdx model, we utilize mdx52/Utrn-/- mice, which exhibit an earlier and more severe phenotype and better reflect the pathology observed in patients with DMD. In particular, echocardiographic assessment of both systolic and diastolic function enables detailed characterization of DMD-associated cardiac pathology. Furthermore, by integrating skeletal muscle functional assessments, blood biomarker analyses, and histopathological evaluations, we comprehensively evaluate disease pathology and pharmacological effects.
Through these integrated evaluation platforms, Axcelead supports the translation of preclinical findings into clinical development.