Respiratory chain inhibition: one more feature to propose MPTP intoxication as a Leigh syndrome model

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• 作者：Barbara Da Costa ; Elodie Dumon…
• 关键词：Mice model ; MPTP ; Respiratory chain ; Mitochondrial diseases
• 刊名：Journal of Bioenergetics and Biomembranes
• 出版年：2016
• 出版时间：October 2016
• 年：2016
• 卷：48
• 期：5
• 页码：483-491
• 全文大小：
• 刊物类别：Chemistry and Materials Science
• 刊物主题：Bioorganic Chemistry; Biochemistry, general; Animal Anatomy / Morphology / Histology; Animal Biochemistry; Organic Chemistry;
• 出版者：Springer US
• ISSN：1573-6881
• 卷排序：48

文摘

1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) intoxicated mice have been widely used to model the loss of dopaminergic neurons. As this treatment leads to basal ganglia degeneration, it was proposed that MPTP mice could be used as a model of Leigh syndrome. However, this mitochondrial pathology is biochemically characterized by a respiratory chain dysfunction. To determine if MPTP can affect in vivo mitochondria function, we measured the activities of mitochondrial respiratory chain complexes in several tissues. Our results show that MPTP affects mainly mitochondrial respiratory chain complex IV, as found in Leigh Syndrome, confirming that acute MPTP intoxicated mice are a good model of Leigh Syndrome.