کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
7634597 | 1494736 | 2016 | 13 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Proteomic analysis of dystrophin deficiency and associated changes in the aged mdx-4cv heart model of dystrophinopathy-related cardiomyopathy
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کلمات کلیدی
موضوعات مرتبط
مهندسی و علوم پایه
شیمی
شیمی آنالیزی یا شیمی تجزیه
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چکیده انگلیسی
The X-linked inherited disorder Duchenne muscular dystrophy is the most frequently inherited neuromuscular disease of childhood. Primary abnormalities in the dystrophin gene trigger progressive skeletal muscle wasting and impaired cardiorespiratory functions. In order to improve our general understanding of the molecular pathogenesis of muscular dystrophy-associated cardiomyopathy and to identify new marker candidates of cardiac changes in dystrophinopathy, we have carried out a comparative proteomic study of the mdx-4cv mouse model of Duchenne muscular dystrophy. The mass spectrometric profiling of whole heart preparations has identified the reduction in the dystrophin-glycoprotein complex and a large variety of secondary changes in the dystrophic heart. Cardiac proteins with a changed abundance were shown to be involved in fibre contraction, energy metabolism, cellular signalling, the cytoskeletal network, the extracellular matrix and the stress response. In the future, the newly identified cardiac proteins may be useful to improve predictive, diagnostic, prognostic or therapy-monitoring approaches in the field of muscular dystrophy and cardiomyopathy.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Journal of Proteomics - Volume 145, 11 August 2016, Pages 24-36
Journal: Journal of Proteomics - Volume 145, 11 August 2016, Pages 24-36
نویسندگان
Sandra Murphy, Paul Dowling, Margit Zweyer, Rustam R. Mundegar, Michael Henry, Paula Meleady, Dieter Swandulla, Kay Ohlendieck,