Fatal breathing dysfunction in a mouse model of Leigh syndrome
نویسندگان
چکیده
منابع مشابه
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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چکیده ندارد.
Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome
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...
متن کاملCorrection for “ Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome , ” by Michele
MEDICAL SCIENCES Correction for “Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome,” by Michele Ferrari, Isha H. Jain, Olga Goldberger, Emanuele Rezoagli, Robrecht Thoonen, Kai-Hung Chen, David E. Sosnovik, Marielle Scherrer-Crosbie, Vamsi K. Mootha, and Warren M. Zapol, which appeared in issue 21, May 23, 2017, of Proc Natl Acad Sci USA (114:E4241–E4250; f...
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Leigh Syndrome (LS) is the most common early-onset, progressive mitochondrial encephalopathy usually leading to early death. The single most prevalent cause of LS is occurrence of mutations in the SURF1 gene, and LS(Surf1) patients show a ubiquitous and specific decrease in the activity of mitochondrial respiratory chain complex IV (cytochrome c oxidase, COX). SURF1 encodes an inner membrane mi...
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ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 2012
ISSN: 0021-9738
DOI: 10.1172/jci62923