A two-site collaborative study of electrical impedance myography for evaluation of disease progression in murine Duchenne muscular dystrophy models

Accessibility to outcome measures that reliably assess disease pathology in preclinical studies are vital, especially for rare disorders such as Duchenne muscular dystrophy (DMD). The non-invasive technique of electrical impedance myography (EIM) has shown potential as an outcome measure in patients and mouse models of neuromuscular disorders. However, these preclinical observations have been limited to a single site. Here, we performed a longitudinal, parallel two-site study to assess the potential of EIM as an outcome measure by using two DMD mouse models - mdx mice on a C57BL/10ScSn/J or DBA/2J genetic background - differing in disease severity, and being hypertrophic and atrophic, respectively, over a ten-month period, in which mice were evaluated monthly. We found that longitudinally acquired phase values were the most reliable, with relatively minor differences between study sites. Histopathology, assessed at 8, 12, 28, or 52 weeks of age correlated well with EIM phase values in both models. Our findings highlight the applicability of EIM as a robust, preclinical outcome measure for DMD.

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Journal
Scientific Reports
Published
2025-09-26
DOI
https://doi.org/10.1038/s41598-025-17137-4
Primary Topic
Muscle activation and electromyography studies
Type
article
Field-Weighted Citation Impact
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article

A two-site collaborative study of electrical impedance myography for evaluation of disease progression in murine Duchenne muscular dystrophy models

Janice A. Nagy, C. Tanganyika-de Winter, Ornella Cappellari, Sarah Engelbeen et al.
Scientific Reports
Muscle activation and electromyography studies
article

A two-site collaborative study of electrical impedance myography for evaluation of disease progression in murine Duchenne muscular dystrophy models

Janice A. Nagy, C. Tanganyika-de Winter, Ornella Cappellari, Sarah Engelbeen, Maaike van Putten, Buket Sonbas Cobb, Brigida Boccanegra, Lisamaura Tulimiero, Sarbesh Pandeya, Kayleigh Putker, Paola Mantuano, Anne-Fleur Schneider, David van de Vijver, Seward B. Rutkove
article en

Abstract

Accessibility to outcome measures that reliably assess disease pathology in preclinical studies are vital, especially for rare disorders such as Duchenne muscular dystrophy (DMD). The non-invasive technique of electrical impedance myography (EIM) has shown potential as an outcome measure in patients and mouse models of neuromuscular disorders. However, these preclinical observations have been limited to a single site. Here, we performed a longitudinal, parallel two-site study to assess the potential of EIM as an outcome measure by using two DMD mouse models - mdx mice on a C57BL/10ScSn/J or DBA/2J genetic background - differing in disease severity, and being hypertrophic and atrophic, respectively, over a ten-month period, in which mice were evaluated monthly. We found that longitudinally acquired phase values were the most reliable, with relatively minor differences between study sites. Histopathology, assessed at 8, 12, 28, or 52 weeks of age correlated well with EIM phase values in both models. Our findings highlight the applicability of EIM as a robust, preclinical outcome measure for DMD.

Scientific ReportsVol. 15(1)
Beth Israel Deaconess Medical Center (US), Leiden University Medical Center (NL), University of Bari Aldo Moro (IT), Harran University (TR)
Duchenne Research Fund, Türkiye Bilimsel ve Teknolojik Araştırma Kurumu
Openalex Percentile: Top 75%
Muscle activation and electromyography studies
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