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Journal article

Diaphragm rescue alone prevents heart dysfunction in dystrophic mice.

Abstract:

Duchenne muscular dystrophy (DMD) is an X-linked recessive disease caused, in most cases, by the complete absence of the 427 kDa cytoskeletal protein, dystrophin. There is no effective treatment, and affected individuals die from respiratory failure and cardiomyopathy by age 30. Here, we investigated whether cardiomyopathy could be prevented in animal models of DMD by increasing diaphragm utrophin or dystrophin expression and thereby restoring diaphragm function. In a transgenic mdx mouse, wh...

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Publication status:
Published

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Publisher copy:
10.1093/hmg/ddq477

Authors


Goyenvalle, A More by this author
Moulton, HM More by this author
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Journal:
Human molecular genetics
Volume:
20
Issue:
3
Pages:
413-421
Publication date:
2011-02-05
DOI:
EISSN:
1460-2083
ISSN:
0964-6906
URN:
uuid:e9f11fff-5f34-4920-971e-fe04b2f7746e
Source identifiers:
106237
Local pid:
pubs:106237

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