Defined neuronal populations drive fatal phenotype 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.
Mammalian neuronal differentiation: early expression of a neuronal phenotype from mouse neural crest cells in a chemically defined culture medium.
We show that mouse neural crest cells cultured in a serum-deprived chemically defined medium on appropriate culture substrata can be induced to express a neuronal phenotype. The uncommitted neural crest cells express a mesenchymal intermediate filament protein such as vimentin, but not the usual neuronal markers such as receptor sites for tetanus toxin or neurofilaments. In the chemically defin...
متن کاملHypoxia 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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ژورنال
عنوان ژورنال: eLife
سال: 2019
ISSN: 2050-084X
DOI: 10.7554/elife.47163