Ultrasonography validation for early alteration of diaphragm echodensity and function in the < i > mdx < /i > mouse model of Duchenne muscular dystrophy

by Antonietta Mele, Paola Mantuano, Adriano Fonzino, Francesco Rana, Roberta Francesca Capogrosso, Francesca Sanarica, Jean-Francois Rolland, Ornella Cappellari, Annamaria De Luca Themdx mouse model of Duchenne muscular dystrophy is characterized by functional and structural alterations of the diaphragm since early stages of pathology, closely resembling patients ’ condition. In recent years, ultrasonography has been proposed as a useful longitudinal non-invasive technique to assessmdx diaphragm dysfunction and evaluate drug efficacy over time. To date, only a few preclinical studies have been conducted. Therefore, an independent validation of this method by different laboratories is needed to increase results reliability and reduce biases. Here, we performed diaphragm ultrasonography in 3- and 6-month-oldmdx mice, the preferred age-window for pharmacology studies. The alteration of diaphragm function over time was measured as diaphragm ultrasound movement amplitude. At the same time points, a first-time assessment of diaphragm echodensity was performed, as an experimental index of progressive loss of contractile tissue. A parallel evaluation of otherin vivo andex vivo dystrophy-relevant readouts was carried out. Both 3- and 6-month-oldmdx mice showed a significant decrease in diaphragm amplitude compared to wild type (wt) mice. This index was well-correlated either within vivo running performance orex vivo isometric tetanic force of isolated diaphragm. In addition, diaphr...
Source: PLoS One - Category: Biomedical Science Authors: Source Type: research