Mitochondrial disease: Rapamycin to the rescue

Mitochondrial disease: Rapamycin to the rescue

Inhibition of the mammalian target of rapamycin (mTOR) pathway with rapamycin attenuates neurological symptoms and extends lifespan in a mouse model of mitochondrial disease. Leigh syndrome is a mitochondrial disease caused by mutations in NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) char...

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Bibliographic Details
Published in:Nature medicine Vol. 19; no. 12; p. 1582
Main Author: Da Silva, Kevin
Format: Journal Article
Language:English
Published: New York Nature Publishing Group 01-12-2013
Subjects:
Biomedical research
Mitochondria
Mutagenesis
Natural products
Studies
Online Access:Get full text
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Summary:Inhibition of the mammalian target of rapamycin (mTOR) pathway with rapamycin attenuates neurological symptoms and extends lifespan in a mouse model of mitochondrial disease. Leigh syndrome is a mitochondrial disease caused by mutations in NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) characterized by stunted growth, myopathy and neurological symptoms, and ultimately death at an early age. Mice lacking Ndufs4 shows many of these features, including neurodegeneration and early death.
ISSN:1078-8956
1546-170X
DOI:10.1038/nm.3428