Animal models of Huntington's disease: implications in uncovering pathogenic mechanisms and developing therapies  

作　　者：Lin-hui WANG Zheng-hong QIN 

机构地区：[1]Departments of Physiology Soochow University School of Medicine, Suzhou 215123, China [2]Departments of Pharmacology, Soochow University School of Medicine, Suzhou 215123, China

出　　处：《Acta Pharmacologica Sinica》2006年第10期1287-1302,共16页中国药理学报（英文版）

基　　金：Project supported by the National Natural Science Foundation of China(No 30370506);by the Specialized Research Fund for the Doctoral Program of Higher Education(No 20050285017).

摘　　要：Huntington＇s disease （HD） is an autosomal dominant neurodegenerative disorder, which is caused by an abnormal expansion of Cytosine Adenine Guanine （CAG） trinucleotide repeat in the gene making huntingtin （Htt）. Despite intensive research efforts devoted to investigate molecular mechanisms of pathogenesis, effective therapy for this devastating disease is still not available at present. The development of various animal models of HD has offered alternative approaches in the study of HD molecular pathology. Many HD models, including chemicalinduced models and genetic models, mimic some aspects of HD symptoms and pathology. To date, however, there is no ideal model which replicates all of the essential features of neuropathology and progressive motor and cognitive impairments of human HD. As a result, our understanding of molecular mechanisms of pathogenesis in HD is still limited. A new model is needed in order to uncover the pathogenesis and to develop novel therapies usefulness and limitations of various animal for HD. In this review we discussed and cellular models of HD in uncovering molecular mechanisms of pathogenesis and developing novel therapies for HD.Huntington＇s disease （HD） is an autosomal dominant neurodegenerative disorder, which is caused by an abnormal expansion of Cytosine Adenine Guanine （CAG） trinucleotide repeat in the gene making huntingtin （Htt）. Despite intensive research efforts devoted to investigate molecular mechanisms of pathogenesis, effective therapy for this devastating disease is still not available at present. The development of various animal models of HD has offered alternative approaches in the study of HD molecular pathology. Many HD models, including chemicalinduced models and genetic models, mimic some aspects of HD symptoms and pathology. To date, however, there is no ideal model which replicates all of the essential features of neuropathology and progressive motor and cognitive impairments of human HD. As a result, our understanding of molecular mechanisms of pathogenesis in HD is still limited. A new model is needed in order to uncover the pathogenesis and to develop novel therapies usefulness and limitations of various animal for HD. In this review we discussed and cellular models of HD in uncovering molecular mechanisms of pathogenesis and developing novel therapies for HD.

关 键 词：Huntington＇s disease HUNTINGTIN NEURODEGENERATION animal models 

分 类 号：R51[医药卫生—内科学]