Non-toxic ubiquitous over-expression of utrophin in the mdx mouse

R Fisher, JM Tinsley, SR Phelps, SE Squire… - Neuromuscular …, 2001 - Elsevier
Duchenne muscular dystrophy (DMD) is an inherited, severe muscle wasting disease
caused by the loss of the cytoskeletal protein, dystrophin. Patients usually die in their late …

Utrophin upregulation in Duchenne muscular dystrophy.

RC Hirst, KJ McCullagh, KE Davies - Acta myologica: myopathies …, 2005 - europepmc.org
Duchenne Muscular Dystrophy (DMD) is a devastating, progressive muscle wasting disease
for which there is currently no effective treatment. DMD is caused by mutations in the …

Microarray analysis of mdx mice expressing high levels of utrophin: therapeutic implications for dystrophin deficiency

D Baban, KE Davies - Neuromuscular Disorders, 2008 - Elsevier
Duchenne Muscular Dystrophy (DMD) is a fatal muscle wasting disorder caused by
dystrophin deficiency. Previous work suggested that increased expression of the dystrophin …

A-utrophin up-regulation in mdx skeletal muscle is independent of regeneration

AP Weir, JE Morgan, KE Davies - Neuromuscular Disorders, 2004 - Elsevier
Duchenne muscular dystrophy is a fatal childhood disease caused by mutations that abolish
the expression of dystrophin in muscle. Utrophin is a paralogue of dystrophin and can …

[HTML][HTML] Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy

AE Deconinck, JA Rafael, JA Skinner, SC Brown… - Cell, 1997 - cell.com
The absence of dystrophin at the muscle membrane leads to Duchenne muscular dystrophy
(DMD), a severe muscle-wasting disease that is inevitably fatal in early adulthood. In …

The role of utrophin in the potential therapy of Duchenne muscular dystrophy

KJ Perkins, KE Davies - Neuromuscular disorders, 2002 - Elsevier
Duchenne muscular dystrophy is an X-linked recessive muscle wasting disease caused by
the absence of the muscle cytoskeletal protein, dystrophin. Dystrophin is a member of the …

Utrophin: a potential replacement for dystrophin?

JM Tinsley, KE Davies - Neuromuscular Disorders, 1993 - Elsevier
This paper reviews the evidence that utrophin, the autosomally encoded protein related to
dystrophin, may be capable of performing the same cellular functions as dystrophin. If this is …

Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice

N Deconinck, J Tinsley, FD Backer, R Fisher, D Kahn… - Nature medicine, 1997 - nature.com
Dystrophin-deficient mice (mdx) expressing a truncated (trc) utrophin transgene show
amelioration of the dystrophic phenotype. Here we report a multifunctional study …

Skeletal muscle-specific expression of a utrophin transgene rescues utrophin-dystrophin deficient mice

JA Rafael, JM Tinsley, AC Potter, AE Deconinck… - Nature …, 1998 - nature.com
Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disease usually
resulting in death of patients by their early twenties1. In contrast, mice lacking dystrophin …

A second promoter provides an alternative target for therapeutic up-regulation of utrophin in Duchenne muscular dystrophy

EA Burton, JM Tinsley, PJ Holzfeind… - Proceedings of the …, 1999 - National Acad Sciences
Duchenne muscular dystrophy (DMD) is an inherited muscle-wasting disease caused by the
absence of a muscle cytoskeletal protein, dystrophin. We have previously shown that …