Development of outcome measures according to dystrophic phenotypes in canine X-linked muscular dystrophy in Japan
| العنوان: | Development of outcome measures according to dystrophic phenotypes in canine X-linked muscular dystrophy in Japan |
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| المؤلفون: | Shin'ichi Takeda, Mutsuki Kuraoka, Yoshitsugu Aoki |
| المصدر: | Experimental Animals |
| بيانات النشر: | Japanese Association for Laboratory Animal Science, 2021. |
| سنة النشر: | 2021 |
| مصطلحات موضوعية: | 0301 basic medicine, Duchenne muscular dystrophy, Male, Pathology, medicine.medical_specialty, osteopontin, Review, Muscle disorder, Motor Activity, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, 0302 clinical medicine, Dogs, Japan, Outcome Assessment, Health Care, medicine, accelerometry, X-linked muscular dystrophy, Animals, Humans, Osteopontin, Muscular dystrophy, General Veterinary, biology, outcome measure, business.industry, Muscle weakness, General Medicine, Young Investigator Award, medicine.disease, canine X-linked muscular dystrophy in Japan (CXMDJ), Gait, Muscular Dystrophy, Duchenne, Disease Models, Animal, 030104 developmental biology, biology.protein, Biomarker (medicine), Animal Science and Zoology, medicine.symptom, business, 030217 neurology & neurosurgery, Biomarkers |
| الوصف: | Duchenne muscular dystrophy (DMD) is an X-linked lethal muscle disorder characterized by primary muscle degeneration. Therapeutic strategies for DMD have been extensively explored, and some are in the stage of human clinical trials. Along with the development of new therapies, sensitive outcome measures are needed to monitor the effects of new treatments. Therefore, we investigated outcome measures such as biomarkers and motor function evaluation in a dystrophic model of beagle dogs, canine X-linked muscular dystrophy in Japan (CXMDJ). Osteopontin (OPN), a myogenic inflammatory cytokine, was explored as a potential biomarker in dystrophic dogs over the disease course. The serum OPN levels of CXMDJ dystrophic dogs were elevated, even in the early disease phase, and this could be related to the presence of regenerating muscle fibers; as such, OPN would be a promising biomarker for muscle regeneration. Next, accelerometry, which is an efficient method to quantify performance in validated tasks, was used to evaluate motor function longitudinally in dystrophic dogs. We measured three-axis acceleration and angular velocity with wireless hybrid sensors during gait evaluations. Multiple parameters of acceleration and angular velocity showed notedly lower values in dystrophic dogs compared with wild-type dogs, even at the onset of muscle weakness. These parameters accordingly decreased with exacerbation of clinical manifestations along with the disease course. Multiple parameters also indicated gait abnormalities in dystrophic dogs, such as a waddling gait. These outcome measures could be applicable in clinical trials of patients with DMD or other muscle disorders. |
| اللغة: | English |
| تدمد: | 1881-7122 1341-1357 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::403a3075c7c2843f0178dbd0728fd88f http://europepmc.org/articles/PMC8614006 |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi.dedup.....403a3075c7c2843f0178dbd0728fd88f |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 18817122 13411357 |
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