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… (More)
DOI: 10.1172/JCI62923


6 Figures and Tables

Slides referencing similar topics