NDLI: Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis.

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Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis.

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Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Miller, Timothy M. Yamanaka, Koji Rizo, Liza Mendell, Jerry R. Arnson, Hannah Hester, Mark Kaspar, Brian K. Umapathi, Priya Kim, Soo H. Gage, Fred H. Cleveland, Don W.
Description	Author Affiliation: Miller TM ( Ludwig Institute for Cancer Research, La Jolla, CA 92093, USA.);
Abstract	Amyotrophic lateral sclerosis (ALS) is a fatal, progressive paralysis arising from the premature death of motor neurons. An inherited form is caused by a dominant mutation in the ubiquitously expressed superoxide dismutase (SOD1). SOD1 mutant expression within motor neurons is a determinant of onset and early disease, and mutant accumulation within microglia accelerates disease progression. Muscle also is a likely primary source for toxicity, because retraction of motor axons from synaptic connections to muscle is among the earliest presymptomatic events. To test involvement of muscle in ALS, viral delivery of transcription-mediated siRNA is shown to suppress mutant SOD1 accumulation within muscle alone but to be insufficient to maintain grip strength, whereas delivery to both motor neurons and muscle is sufficient. Use of a deletable mutant gene to diminish mutant SOD1 from muscle did not affect onset or survival. Finally, follistatin expression encoded by adeno-associated virus chronically inhibited myostatin and produced sustained increases in muscle mass, myofiber number, and fiber diameter, but these increases did not affect survival. Thus, SOD1-mutant-mediated damage within muscles is not a significant contributor to non-cell-autonomous pathogenesis in ALS, and enhancing muscle mass and strength provides no benefit in slowing disease onset or progression.
ISSN	00278424
e-ISSN	10916490
Journal	Proceedings of the National Academy of Sciences of the United States of America
Issue Number	51
Volume Number	103
Language	English
Publisher	National Academy of Sciences
Publisher Date	2006-12-01
Publisher Place	United States
Access Restriction	Open
Subject Keyword	Amyotrophic Lateral Sclerosis Genetics Metabolism Motor Neurons Muscle, Skeletal Mutation Superoxide Dismutase Animals Cell Line Dependovirus Disease Progression Follistatin Genetic Vectors Hand Strength Physiology Mice Cytology RNA, Small Interfering Comparative Study Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Multidisciplinary
Content Type	Text
Resource Type	Article
Subject	Multidisciplinary

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