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Naturally occurring utrophin correlates with disease severity in Duchenne muscular dystrophy

4 Apr 2006

Abstract excerpt

Although there is good experimental data that utrophin, the autosomal analog of dystrophin, can ameliorate the phenotype in dystrophinopathies, there is scant evidence from human data to support this hypothesis. We investigated in diagnostic muscle biopsies from 16 patients with Duchenne muscular dystrophy (DMD) the level of utrophin expression using quantitative immunoblot analysis. In 13 of 16 patients, in whom...

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PMID: 16595608
DOI: 10.1093/hmg/ddl083

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Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Naturally occurring utrophin correlates with disease severity in Duchenne muscular dystrophyPMID 16595608Read ↗

Concordant utrophin upregulation in phenotypically discordant DMD/BMD brothers.Semantic proximity Read ↗

The dystrophin-related protein, utrophin, is expressed on the sarcolemma of regenerating human skeletal muscle fibres in dystrophies and inflammatory myopathiesSemantic proximity Read ↗

A- and B-utrophin Have Different Expression Patterns and Are Differentially Up-regulated in mdx MuscleSemantic proximity Read ↗

Dystrophin- and Utrophin-Based Therapeutic Approaches for Treatment of Duchenne Muscular Dystrophy: A Comparative ReviewSemantic proximity Read ↗

Biglycan recruits utrophin to the sarcolemma and counters dystrophic pathology in mdx miceSemantic proximity Read ↗

Undetectable dystrophin can still result in a relatively benign phenotype of dystrophinopathy.Semantic proximity Read ↗

Sarcolemmal nNOS anchoring reveals a qualitative difference between dystrophin and utrophinSemantic proximity Read ↗

Distal mdx muscle groups exhibiting up-regulation of utrophin and rescue of dystrophin-associated glycoproteins exemplify a protected phenotype in muscular dystrophy.Semantic proximity Read ↗

Increased utrophin expression in healthy and DMD patient derived myoblasts in response to ERK1/2 and EZH2 inhibitor treatmentSemantic proximity Read ↗

Daily Treatment with SMTC1100, a Novel Small Molecule Utrophin Upregulator, Dramatically Reduces the Dystrophic Symptoms in the mdx MouseSemantic proximity Read ↗

The potential of utrophin and dystrophin combination therapies for Duchenne muscular dystrophySemantic proximity Read ↗

Heregulin ameliorates the dystrophic phenotype in mdx mice.Semantic proximity Read ↗

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