DMD模型鼠mdx小鼠线粒体损伤的研究进展  

Progress of research into mitochondrial injury in mdx mice

作　　者：高天 董健健 喻绪恩 GAO Tian;DONG Jianjian;YU Xuen(Institute of Neurology,Anhui University of Chinese Medicine,Hefei 230038,China;Affiliated Hospital of the Neurology Institute,Anhui University of Chinese Medicine,Hefei 230061,China)

机构地区：[1]安徽中医药大学神经病学研究所,合肥230038 [2]安徽中医药大学神经病学研究所附属医院,合肥230061

出　　处：《中国实验动物学报》2024年第6期793-798,共6页Acta Laboratorium Animalis Scientia Sinica

基　　金：安徽省高校自然科学研究重点项目(KJ2021A0550);安徽中医药大学临床科研自然重大项目(2021sfylc02)。

摘　　要：Duchenne肌营养不良(Duchenne muscular dystrophy,DMD)是一种由编码抗肌萎缩蛋白的Dystrophin基因突变导致的致死性、进行性、X连锁隐性遗传肌肉疾病。目前,DMD尚无治愈手段,临床研究进展缓慢,动物模型的建立对DMD的实验研究作用越来越重要。结合研究发现,mdx小鼠具有DMD患者相同的发病机制,广泛应用于DMD病理机制和新药开发的研究中。线粒体损伤是DMD重要的病理机制之一,对线粒体的保护是DMD的潜在治疗靶点,因此探讨mdx小鼠与线粒体损伤的关系具有重要意义。本文就近年来DMD模型鼠mdx小鼠线粒体损伤的研究进展进行综述,为相关实验提供参考。Duchenne muscular dystrophy(DMD)is a lethal,progressive,X⁃linked recessive hereditary muscle disease caused by a mutation in the gene encoding dystrophin.Currently,no cure for DMD is available,and clinical research is progressing slowly.The establishment of animal models is becoming increasingly important for experimental research on DMD.Following the research findings that mdx mice have the same pathogenesis as DMD patients,this model is widely used in the study of DMD pathogenesis and drug development.Mitochondrial injury is one of the most important pathological mechanisms of DMD,and mitochondrial protection is a potential therapeutic strategy for DMD,and thus it is significant to study mitochondrial injury in mdx mice.This article reviews the progress of research into mitochondrial injury in DMD model mdx mice in recent years to provide a reference for related experiments.

关 键 词：DUCHENNE肌营养不良 线粒体损伤 动物模型 mdx小鼠 

分 类 号：Q95-33[生物学—动物学]