Excitotoxicity and mitochondrial pathogenesis and animal models of Huntington’s disease
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1.Research Center for Translational Medicine & Key Laboratory of Arrhythmias of the Ministry of Education of China, Shanghai East Hospital, Tongji University School of Medicine, Shanghai 200120, China. 2. Rehabilitation Center, Qilu Children’s Hospital, Shandong University, Jinan 250022. 3. National Clinic and Medicine Research Institute for Geriatric Diseases, the First Affiliated Hospital, Gannan University of Medical Sciences, Ganzhou 341000

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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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History
  • Received:November 25,2020
  • Revised:
  • Adopted:
  • Online: September 10,2021
  • Published: