Excitotoxicity and mitochondrial pathogenesis and animal models of Huntington’s disease
Received:November 25, 2020  
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DOI:10. 3969 / j.issn.1005-4847. 2021. 04. 018
KeyWord:Huntington’s disease; animal models; neurons; mitochondria; excitatory amino acid
              
AuthorInstitution
唐文洁 同济大学附属东方医院转化医学研究中心,教育部心律失常重点实验室,上海
黄艳 山东大学齐鲁 儿童医院康复科,济南
王力峰 赣南医学院附属第一医院国家老年病临床研究中心,江西 赣州
魏佑震 同济大学附属东方医院转化医学研究中心,教育部心律失常重点实验室,上海
谢元云 赣南医学院附属第一医院国家老年病临床研究中心,江西 赣州
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Abstract:
      Huntington’ s disease ( HD) is an inherited autosomal dominant and progressive neurodegenerative disease with locomotor, cognitive, and psychiatric functional deficits. Currently, there is no cure for HD, and the clinical benefit is greatly limited for some treatments available for controlling HD symptoms. In 1993, it was discovered that HD is caused by a single Huntingtin gene (HTT) mutation with 36 CAG repeats and or more, which translates to a mutated polyglutamine, Resulting in neuronal excitotoxicity and abnormal mitochondrial energy metabolism, and eventually neurodegeneration in the cerebral cortex and basal nuclei. To better understand the pathogenesis mechanisms of HD and explore therapeutic interventions, it is important to create HD animal models. In this review, we will introduce HD animal models based on neuropathology, and explore the molecular biology of HD such as neural excitotoxicity and mitochondrial toxicity.
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