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Daily treatment with SMTC1100, a novel small molecule utrophin upregulator, dramatically reduces the dystrophic symptoms in the mdx mouse.

Abstract:

BACKGROUND: Duchenne muscular dystrophy (DMD) is a lethal, progressive muscle wasting disease caused by a loss of sarcolemmal bound dystrophin, which results in the death of the muscle fibers leading to the gradual depletion of skeletal muscle. There is significant evidence demonstrating that increasing levels of the dystrophin-related protein, utrophin, in mouse models results in sarcolemmal bound utrophin and prevents the muscular dystrophy pathology. The aim of this work was to develop a s...

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Publication status:
Published online

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Publisher copy:
10.1371/journal.pone.0019189

Authors


Tinsley, JM More by this author
More by this author
Institution:
University of Oxford
Wilkes, FJ More by this author
More by this author
Institution:
University of Oxford
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Medical Research Council More from this funder
Muscular Dystrophy Campaign More from this funder
Muscular Dystrophy Association USA More from this funder
Association Francaise contre les Myopathies More from this funder
Telethon Italy More from this funder
Publisher:
Public Library of Science Publisher's website
Journal:
PloS one Journal website
Volume:
6
Issue:
5
Pages:
Article: e19189
Publication date:
2011-01-05
DOI:
EISSN:
1932-6203
ISSN:
1932-6203
URN:
uuid:ed989d83-882f-490c-b5ff-d650858e7db3
Source identifiers:
146689
Local pid:
pubs:146689

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