کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
6041726 | 1189316 | 2012 | 12 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
علوم اعصاب تکاملی
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
The genetic defect of mdx mice resembles that of Duchenne muscular dystrophy, although their functional performance and life expectancy is nearly normal. By contrast, mice lacking utrophin and dystrophin (mdx/utrn â/â) are severely affected and die prematurely. Mice with one utrophin allele (mdx/utrn +/â) are more severely affected than mdx mice, but outlive mdx/utrn â/â mice. We subjected mdx/utrn +/+, +/â, â/â and wild type males to a 12Â week functional test regime of four different functional tests. Mdx/utrn +/+ and +/â mice completed the regime, while mdx/utrn â/â mice died prematurely. Mdx/utrn +/â mice performed significantly worse compared to mdx/utrn +/+ mice in functional tests. Creatine kinase levels, percentage of fibrotic/necrotic tissue, morphology of neuromuscular synapses and expression of biomarker genes were comparable, whereas mdx/utrn +/â and â/â mice had increased levels of regenerating fibers. This makes mdx/utrn +/â mice valuable for testing the benefit of potential therapies on muscle function parameters.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neuromuscular Disorders - Volume 22, Issue 5, May 2012, Pages 406-417
Journal: Neuromuscular Disorders - Volume 22, Issue 5, May 2012, Pages 406-417
نویسندگان
Maaike van Putten, Darshan Kumar, Margriet Hulsker, Willem M.H. Hoogaars, Jaap J. Plomp, Annemarieke van Opstal, Maarten van Iterson, Peter Admiraal, Gert-Jan B. van Ommen, Peter A.C. 't Hoen, Annemieke Aartsma-Rus,