Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains

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• 作者：Maaike van Putten^a ; Darshan Kumar^a ; Margriet Hulsker^a ; Willem M.H. Hoogaars^a ; Jaap J. Plomp^b ; Annemarieke van Opstal^a ; Maarten van Iterson^a ; Peter Admiraal^a ; Gert-Jan B. van Ommen^a ; Peter A.C. ‘ ; t Hoen^a ; Annemieke Aartsma-Rus^a ; ^{a.m.rus@lumc.nl}
• 关键词：Duchenne muscular dystrophy ; Dystrophin ; Utrophin ; Functional tests ; Histopathology ; Biomarkers
• 刊名：Neuromuscular Disorders
• 出版年：2012
• 期刊代码：203_09608966
• 类别：med
• 出版时间：May, 2012
• 卷：22
• 期：5
• 页码：406-417
• 文件大小：1321 K

摘要

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 鈭?鈭? are severely affected and die prematurely. Mice with one utrophin allele (mdx/utrn +/鈭? are more severely affected than mdx mice, but outlive mdx/utrn 鈭?鈭?mice. We subjected mdx/utrn +/+, +/鈭? 鈭?鈭?and wild type males to a 12 week functional test regime of four different functional tests. Mdx/utrn +/+ and +/鈭?mice completed the regime, while mdx/utrn 鈭?鈭?mice died prematurely. Mdx/utrn +/鈭?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 +/鈭?and 鈭?鈭?mice had increased levels of regenerating fibers. This makes mdx/utrn +/鈭?mice valuable for testing the benefit of potential therapies on muscle function parameters.