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Complex transcription of the utrophin gene: A study of short utrophin isoforms
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Actin conformation dynamics precede force generation in cytotoxic T lymphocytes
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Downstream utrophin enhancer is required for expression of utrophin in skeletal muscle
The Journal of Gene Medicine, 2008AbstractBackgroundDuchenne muscular dystrophy is caused by the absence of the muscle cytoskeletal protein dystrophin. Utrophin is an autosomal homologue of dystrophin, and overexpression of utrophin is expected to compensate for the dystrophin deficit.
Jun, Tanihata +4 more
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A study of short utrophin isoforms in mice deficient for full-length utrophin
Mammalian Genome, 2003Utrophin can functionally replace dystrophin in dystrophin-deficient muscle and may have a role in a therapeutic strategy for Duchenne muscular dystrophy. This has resulted in many investigations of the full-length muscle form of utrophin; however, the short utrophins and non-muscle forms have been relatively neglected, partly because they are ...
Jimenez-Mallebrera C +3 more
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Utrophin: A potential replacement for dystrophin?
Neuromuscular Disorders, 1993This 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 the case, it may be possible to modify the regulation of utrophin expression as an alternative route to dystrophin gene therapy for sufferers of DMD and/or BMD.
J M, Tinsley, K E, Davies
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Nature Genetics, 1998
Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disease usually resulting in death of patients by their early twenties. In contrast, mice lacking dystrophin (Dmd(mdx)), appear physically normal despite their underlying muscle pathology.
J A, Rafael +4 more
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Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disease usually resulting in death of patients by their early twenties. In contrast, mice lacking dystrophin (Dmd(mdx)), appear physically normal despite their underlying muscle pathology.
J A, Rafael +4 more
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Utrophin is a calpain substrate in muscle cells
Muscle & Nerve, 2006AbstractCalpains are Ca2+‐dependent cytosolic cysteine proteases that participate in the pathology of Duchenne muscular dystrophy (DMD). Utrophin is a functional homolog of dystrophin that partially compensates for dystrophin deficiency in myofibers of mdx mice.
Isabelle, Courdier-Fruh +1 more
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Utrophin muscles in on the action
Nature Medicine, 1997Overexpressing utrophin in muscles may prove a promising strategy for treating Duchenne muscular dystrophy but many questions remain unanswered.
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