Journal article
Dp71 can restore the dystrophin-associated glycoprotein complex in muscle but fails to prevent dystrophy
Nature genetics, Vol.8(4), pp.333-339
12/1994
DOI: 10.1038/ng1294-333
PMID: 7894482
Abstract
Two lines of transgenic mdx mice have been generated that express a 71 kD non-muscle isoform of dystrophin (Dp71) in skeletal muscle. This isoform contains the cysteine-rich and Oterminal domains of dystrophin, but lacks the N-terminal actin-binding and central spectrin-like repeat domains. Dp71 was associated with the sarcolemma membrane, where it restored normal expression and localization of all members of the dystrophin-associated glycoprotein complex. However, the skeletal muscle pathology of the transgenic mdx mice remained severe. These results indicate that the dystrophin C terminus cannot function independently to prevent dystrophic symptoms and confirms predictions based on patient data that both the N and C-terminal domains are required for normal dystrophin function.
Details
- Title: Subtitle
- Dp71 can restore the dystrophin-associated glycoprotein complex in muscle but fails to prevent dystrophy
- Creators
- Gregory A CoxYoshihide SunadaKevin P CampbellJeffrey S Chamberlain
- Resource Type
- Journal article
- Publication Details
- Nature genetics, Vol.8(4), pp.333-339
- DOI
- 10.1038/ng1294-333
- PMID
- 7894482
- ISSN
- 1061-4036
- eISSN
- 1546-1718
- Language
- English
- Date published
- 12/1994
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Iowa Neuroscience Institute
- Record Identifier
- 9984020742202771
Metrics
12 Record Views