mTOR Inhibition Alleviates Mitochondrial Disease in a Mouse Model of Leigh Syndrome-Reference-Cited by-同舟云学术

mTOR Inhibition Alleviates Mitochondrial Disease in a Mouse Model of Leigh Syndrome

Published:2013-12-20 Issue:6165 Volume:342 Page:1524-1528
ISSN:0036-8075
Container-title:Science
language:en
Short-container-title:Science

Author:

Johnson Simon C.¹,Yanos Melana E.¹²,Kayser Ernst-Bernhard³,Quintana Albert⁴,Sangesland Maya¹,Castanza Anthony¹,Uhde Lauren¹,Hui Jessica¹,Wall Valerie Z.¹,Gagnidze Arni¹,Oh Kelly¹,Wasko Brian M.¹,Ramos Fresnida J.¹,Palmiter Richard D.⁴,Rabinovitch Peter S.¹,Morgan Philip G.³,Sedensky Margaret M.³,Kaeberlein Matt¹

Affiliation:

1. Department of Pathology, University of Washington, Seattle, WA 98195, USA.

2. Department of Psychology, University of Washington, Seattle, WA 98195, USA.

3. Anesthesiology and Pain Medicine, Seattle Children’s Hospital, Seattle, WA 98105, USA.

4. Howard Hughes Medical Institute and Department of Biochemistry, University of Washington, Seattle, WA 98195, USA.

Abstract

More from mTOR Leigh syndrome is a rare, untreatable, inherited neurodegenerative disease in children that is caused by functional disruption of mitochondria, the cell's energy-producing organelles. Johnson et al. (p. 1524 , published online 14 November; see Perspective by

Vafai and Mootha

) show that rapamycin, a drug used clinically as an immunosuppressant and for treatment of certain cancers, delayed the onset and progression of neurological symptoms in a mouse model of Leigh syndrome and significantly extended survival of the animals. Rapamycin inhibits the so-called “mTOR” signaling pathway, which is currently under intense study because it plays a contributory role in many common diseases.

Publisher

American Association for the Advancement of Science (AAAS)

Subject

Multidisciplinary

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