- Abstract:
-
Duchenne muscular dystrophy (DMD) is caused by loss of dystrophin protein, leading to progressive muscle weakness and premature death due to respiratory and/or cardiac complications. Cardiac involvement is characterized by progressive dilated cardiomyopathy, decreased fractional shortening and metabolic dysfunction involving reduced metabolism of fatty acids-the major cardiac metabolic substrate. Several mouse models have been developed to study molecular and pathological consequences of dyst...
Expand abstract - Publication status:
- Published
- Peer review status:
- Peer reviewed
- Version:
- Publisher's Version
- Files:
-
-
(pdf, 3.6MB)
-
- Publisher copy:
- 10.1093/hmg/ddy346
-
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- Publisher:
- Oxford University Press Publisher's website
- Journal:
- Human Molecular Genetics Journal website
- Volume:
- 28
- Issue:
- 3
- Pages:
- 396–406
- Publication date:
- 2018-10-02
- Acceptance date:
- 2018-09-21
- DOI:
- EISSN:
-
1460-2083
- ISSN:
-
0964-6906
- Pubs id:
-
pubs:923772
- URN:
-
uri:c36deec4-3259-4a35-adf8-7622f9d6708b
- UUID:
-
uuid:c36deec4-3259-4a35-adf8-7622f9d6708b
- Local pid:
- pubs:923772
- Language:
- English
- Copyright holder:
- Betts et al.
- Copyright date:
- 2018
- Notes:
- © The Author(s) 2018. Published by Oxford University Press. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
Journal article
Cmah-dystrophin deficient mdx mice display an accelerated cardiac phenotype that is improved following peptide-PMO exon skipping treatment
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