Behavioral, Biochemical and Pathological Characterization of a new MDX Mouse Model of Duchenne Muscular Dystrophy

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dc.contributor.authorWang, Fengjiaoen_US
dc.contributor.authorWen, Jingen_US
dc.contributor.authorGuo, Baojianen_US
dc.contributor.authorWu, Liangmiaoen_US
dc.contributor.authorLiu, Zhengen_US
dc.contributor.authorZaijun, Zhangen_US
dc.date.accessioned2020-11-18T10:29:12Z
dc.date.available2020-11-18T10:29:12Z
dc.date.issued2020-06
dc.description.abstractBackground Duchenne muscular dystrophy (DMD) is an X-linked inherited neuromuscular disorder due tomutations in the dystrophin gene. Animal models that accurately reflect pathological conditions and diseasecharacteristics are key factors in the discovery and development of new anti-DMD drugs.Aim Here, we evaluated motor behavior, pathological and biochemical characters of a new DMD mouse modelbuilt up by the Nanjing Biomedical Research Institute of Nanjing University (NBRI).Methods The pole test and open-field test were used to assess the movement disorders in DMD mouse model.The gastrocnemius (GAS), biceps, triceps, soleus, and tibialis anterior muscles of mice were subjected to weight analysis to evaluate the skeletal muscle pseudohypertrophy. Meanwhile, immunofluorescence andWestern blotting were used to detect the expression of dystrophin in the GAS. Serum levels of creatine kinase(CK) and lactate dehydrogenase (LDH) that accurately reflect muscle damage were detected. Masson stainingwas used to evaluate the fibrosis of GAS and diaphragm (DIA).Results The novel DMD mouse showed significant behavioral disorders and exhibited high serum levels of CKand LDH. Western blotting and immunofluorescence staining showed decreased significantly with dystrophinlevel in the GAS. Besides, the mdx mouse of DMD developed fibrosis in both GAS and DIA.Conclusion Taken together, our results indicated that the behavioral, biochemical and pathologicalcharacterization of the mdx mouse model is similar to human DMD. This mdx mouse model may provideinsights into the pathophysiology of DMD and the effects of anti-DMD drugs.en_US
dc.identifier.affiliationsInstitute of New Drug Research; International Cooperative Laboratory of Traditional Chinese Medicine Modernization and Innovative Drug Development of Chinese Ministry of Education, Jinan University College of Pharmacy, Guangzhou 510632, Chinaen_US
dc.identifier.affiliationsSchool of Stomatology and Medicine & Foshan Stomatology Hospital, Foshan University, Foshan 528000, Chinaen_US
dc.identifier.affiliationsFoshan Magpie Pharmaceuticals Co., LTD, Foshan, Chinaen_US
dc.identifier.citationWang Fengjiao, Wen Jing, Guo Baojian, Wu Liangmiao, Liu Zheng, Zaijun Zhang. Behavioral, Biochemical and Pathological Characterization of a new MDX Mouse Model of Duchenne Muscular Dystrophy. Journal of Pharmaceutical and Biomedical Sciences. 2020 Jun; 10(6): 119-128en_US
dc.identifier.issn2230-7885
dc.identifier.placeIndiaen_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/215721
dc.languageenen_US
dc.publisherLawarence Press Pvt. Ltd.en_US
dc.relation.issuenumber6en_US
dc.relation.volume10en_US
dc.source.urihttps://lawarencepress.com/ojs/index.php/JPBMS/article/view/615/pdf_299en_US
dc.subjectDuchenne muscular dystrophyen_US
dc.subjectBehavioral disorderen_US
dc.subjectCreatine kinaseen_US
dc.subjectDystrophinen_US
dc.subjectFibrosisen_US
dc.titleBehavioral, Biochemical and Pathological Characterization of a new MDX Mouse Model of Duchenne Muscular Dystrophyen_US
dc.typeJournal Articleen_US
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