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The emperor's new dystrophin: Finding sense in the noise
Journal article   Peer reviewed

The emperor's new dystrophin: Finding sense in the noise

S.D. Wilton, R.N. Veedu and S. Fletcher
Trends in Molecular Medicine, Vol.21(7), pp.417-426
2015
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Abstract

Targeted dystrophin exon removal is a promising therapy for Duchenne muscular dystrophy (DMD); however, dystrophin expression in some reports is not supported by the associated data. As in the account of ‘The Emperor's New Clothes’, the validity of such claims must be questioned, with critical re-evaluation of available data. Is it appropriate to report clinical benefit and induction of dystrophin as dose dependent when the baseline is unclear? The inability to induce meaningful levels of dystrophin does not mean that dystrophin expression as an end point is irrelevant, nor that induced exon skipping as a strategy is flawed, but demands that drug safety and efficacy, and study parameters be addressed, rather than questioning the strategy or the validity of dystrophin as a biomarker.

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Citation topics
1 Clinical & Life Sciences
1.255 Musculoskeletal Disorders
1.255.628 Duchenne Muscular Dystrophy
Web Of Science research areas
Biochemistry & Molecular Biology
Cell Biology
Medicine, Research & Experimental
ESI research areas
Molecular Biology & Genetics
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