Abstract:
:Duchenne muscular dystrophy is a devastating muscular dystrophy of childhood. Mutations in the dystrophin gene destroy the link between the internal muscle filaments and the extracellular matrix, resulting in severe muscle weakness and progressive muscle wasting. There is currently no cure and, whilst palliative treatment has improved, affected boys are normally confined to a wheelchair by 12 years of age and die from respiratory or cardiac complications in their twenties or thirties. Therapies currently being developed include mutation-specific treatments, DNA- and cell-based therapies, and drugs which aim to modulate cellular pathways or gene expression. This review aims to provide an overview of the different therapeutic approaches aimed at reconstructing the dystrophin-associated protein complex, including restoration of dystrophin expression and upregulation of the functional homologue, utrophin.
journal_name
Exp Physioljournal_title
Experimental physiologyauthors
Fairclough RJ,Bareja A,Davies KEdoi
10.1113/expphysiol.2010.053025subject
Has Abstractpub_date
2011-11-01 00:00:00pages
1101-13issue
11eissn
0958-0670issn
1469-445Xpii
expphysiol.2010.053025journal_volume
96pub_type
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