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mdx 小鼠的跖屈挛缩。

Plantarflexion contracture in the mdx mouse.

机构信息

Program in Physical Therapy and Rehabilitation Sciences, School of Medicine, University of Minnesota, Minneapolis, USA.

出版信息

Am J Phys Med Rehabil. 2010 Dec;89(12):976-85. doi: 10.1097/PHM.0b013e3181fc7c9e.

DOI:10.1097/PHM.0b013e3181fc7c9e
PMID:21403594
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3392333/
Abstract

OBJECTIVE

Contractures are a major clinical issue for patients with muscular dystrophies. However, it is unknown whether contractures are present in the widely used mdx mouse model of Duchenne muscular dystrophy. Therefore, the objectives of this study were to develop methods to measure muscle contractures in mice, to determine whether plantarflexion contractures are present in mdx mice, and to analyze the composition of the major muscles involved.

DESIGN

Hindlimbs of eight wild type and six mdx mice were assessed every 2 wks during the course of a 12-wk study. Assessments included range of motion and in vivo torques about the ankle. At the end of the study, mice were euthanized, and muscles were analyzed for composition.

RESULTS

The mdx mice had ∼10 degrees less dorsiflexion, increased passive torque moving the ankle into dorsiflexion, and an increased passive-to-active torque ratio relative to wild type mice. Gastrocnemius muscle composition alterations included increased wet mass, decreased protein content, and increased collagen.

CONCLUSIONS

The results indicate that mdx mice have plantarflexion contractures similar to those seen in children with Duchenne muscular dystrophy. In future studies, these measures can be used to assess strategies to slow the progression of contractures that occur with muscular dystrophies.

摘要

目的

肌肉萎缩症患者存在严重的临床挛缩问题。但目前尚不清楚杜氏肌营养不良症的常用 mdx 小鼠模型是否存在挛缩。因此,本研究的目的是开发测量小鼠肌肉挛缩的方法,确定 mdx 小鼠是否存在跖屈挛缩,并分析主要受累肌肉的组成。

设计

在为期 12 周的研究过程中,每隔 2 周评估 8 只野生型和 6 只 mdx 小鼠的后肢。评估包括踝关节的运动范围和体内扭矩。研究结束时,处死小鼠并分析肌肉组成。

结果

mdx 小鼠的背屈度减少约 10 度,被动扭矩使踝关节进入背屈增加,与野生型小鼠相比,被动到主动扭矩比增加。比目鱼肌的组成变化包括湿重增加、蛋白含量减少和胶原增加。

结论

结果表明,mdx 小鼠存在类似杜氏肌营养不良症患儿的跖屈挛缩。在未来的研究中,这些措施可用于评估减缓与肌肉萎缩症相关的挛缩进展的策略。

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本文引用的文献

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Effects of prednisolone on skeletal muscle contractility in mdx mice.泼尼松龙对mdx小鼠骨骼肌收缩性的影响。
Muscle Nerve. 2009 Sep;40(3):443-54. doi: 10.1002/mus.21327.
2
Adaptive and nonadaptive responses to voluntary wheel running by mdx mice.mdx小鼠对自愿性转轮运动的适应性和非适应性反应。
Muscle Nerve. 2008 Oct;38(4):1290-303. doi: 10.1002/mus.21141.
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Prevention of muscle fibrosis and improvement in muscle performance in the mdx mouse by halofuginone.卤夫酮对mdx小鼠肌肉纤维化的预防及肌肉性能的改善
Neuromuscul Disord. 2008 Nov;18(11):857-68. doi: 10.1016/j.nmd.2008.06.386. Epub 2008 Jul 30.
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Dystrophin-deficient mdx mice display a reduced life span and are susceptible to spontaneous rhabdomyosarcoma.肌营养不良蛋白缺陷的mdx小鼠寿命缩短,且易患自发性横纹肌肉瘤。
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A comparison of factors associated with collagen metabolism in different skeletal muscles from dystrophic (mdx) mice: impact of pirfenidone.对营养不良(mdx)小鼠不同骨骼肌中与胶原蛋白代谢相关因素的比较:吡非尼酮的影响
Muscle Nerve. 2007 Feb;35(2):208-16. doi: 10.1002/mus.20681.
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The muscular dystrophies: from genes to therapies.肌营养不良症:从基因到疗法。
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Compositional analysis of muscle in boys with Duchenne muscular dystrophy using MR imaging.利用磁共振成像对杜兴氏肌营养不良症男孩的肌肉进行成分分析。
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Progression of kyphosis in mdx mice.
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Longitudinal pathologic study of the gastrocnemius muscle group in mdx mice.mdx小鼠腓肠肌群的纵向病理学研究
Acta Neuropathol. 2004 Jan;107(1):27-34. doi: 10.1007/s00401-003-0773-3. Epub 2003 Oct 7.
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Management of progressive muscular dystrophy in childhood.儿童进行性肌营养不良的管理
JAMA. 1963 Apr 13;184:89-96. doi: 10.1001/jama.1963.03700150043007.