Wang, FengjiaoWen, JingGuo, BaojianWu, LiangmiaoLiu, ZhengZaijun, Zhang2020-11-182020-11-182020-06Wang 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): 119-1282230-7885http://imsear.searo.who.int/handle/123456789/215721Background 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.Duchenne muscular dystrophyBehavioral disorderCreatine kinaseDystrophinFibrosisJournal ArticleIndiaInstitute 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, ChinaSchool of Stomatology and Medicine & Foshan Stomatology Hospital, Foshan University, Foshan 528000, ChinaFoshan Magpie Pharmaceuticals Co., LTD, Foshan, China