Histological parameters for the quantitative assessment of muscular dystrophy in the mdx-mouse

被引：328

作者：

Briguet, A ^{[1
]}

Courdier-Fruh, I ^{[1
]}

Foster, M ^{[1
]}

Meier, T ^{[1
]}

Magyar, JP ^{[1
]}

机构：

[1] MyoContract Ltd, CH-4410 Liestal, Switzerland

来源：

NEUROMUSCULAR DISORDERS | 2004年 / 14卷 / 10期

关键词：

Duchenne muscular dystrophy; mdx-mouse; histology; variance coefficient;

D O I：

10.1016/j.nmd.2004.06.008

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Duchenne muscular dystrophy is a severe X-linked hereditary disease caused by the absence of functional dystrophin. The dystrophin-deficient mdx-mouse strain is a widely used animal model for dystrophin-deficiency. Several therapeutic approaches for muscular dystrophy have been proposed by different laboratories. In order to compare the efficacy of these therapies in the mdx-mouse, it is essential to implement standardized protocols for the assessment of functional and histological parameters in this mouse model. Here, we determine that the minimal 'Feret's diameter' is a geometrical parameter that allows for reliable measure of muscle fiber cross-sectional size. Using this geometrical parameter we calculate variance coefficients of the muscle fiber size and provide reference values for the quantitative assessment of dystrophic symptoms in frequently investigated muscles of wild-type and mdx-mouse. In addition, we compare the variance coefficients of the muscle fiber size with the percentage of muscle fibers with centralized nuclei; another histological hallmark of muscular dystrophy. (C) 2004 Elsevier B.V. All rights reserved.

引用

页码：675 / 682

页数：8

共 27 条

[1]

HISTOPATHOLOGICAL CHANGES IN DUCHENNE MUSCULAR DYSTROPHY [J].