Acarbose suppresses symptoms of mitochondrial disease in a mouse model of Leigh Syndrome
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چکیده
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Fatal breathing dysfunction in a mouse model of Leigh syndrome.
Leigh syndrome (LS) is a subacute necrotizing encephalomyelopathy with gliosis in several brain regions that usually results in infantile death. Loss of murine Ndufs4, which encodes NADH dehydrogenase (ubiquinone) iron-sulfur protein 4, results in compromised activity of mitochondrial complex I as well as progressive neurodegenerative and behavioral changes that resemble LS. Here, we report the...
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The most common pediatric mitochondrial disease is Leigh syndrome, an episodic, subacute neurodegeneration that can lead to death within the first few years of life, for which there are no proven general therapies. Mice lacking the complex I subunit, Ndufs4, develop a fatal progressive encephalopathy resembling Leigh syndrome and die at ≈60 d of age. We previously reported that continuously bre...
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ژورنال
عنوان ژورنال: Innovation in Aging
سال: 2020
ISSN: 2399-5300
DOI: 10.1093/geroni/igaa057.3271