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运动的mdx小鼠中更大的结肠直肠激活表型

Greater Colo-Rectal Activation Phenotype in Exercised mdx Mice.

作者信息

Nearing Marie, Novak James, Partridge Terence

机构信息

Children's National Health System, Children's Research Institute, Center for Genetic Medicine Research, Washington DC, United States.

Center for Genetic Medicine Research, Children's National Health System, Washington DC, United States.

出版信息

PLoS Curr. 2018 May 2;10:ecurrents.md.230ed3d6559b171e10279fc16e9ebef3. doi: 10.1371/currents.md.230ed3d6559b171e10279fc16e9ebef3.

DOI:10.1371/currents.md.230ed3d6559b171e10279fc16e9ebef3
PMID:29862132
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5959732/
Abstract

INTRODUCTION

Duchenne Muscular Dystrophy is a genetic disease that is caused by a deficiency of dystrophin protein. Both Duchenne Muscular Dystrophy patients and dystrophic mice suffer from intestinal dysfunction.

METHODS

The present study arose from a chance observation of differences in fecal output of dystrophic vs. normal mice during 20-minutes of forced continuous treadmill exercise. Here, we report on the effects of exercise on fecal output in two different dystrophic mutants and their normal background control strains. All fecal materials evacuated during exercise were counted, dried and weighed.

RESULTS

Mice of both mutant dystrophic strains produced significantly more fecal material during the exercise bout than the relevant control strains.

ISCUSSION

We propose that exercise--induced Colo--Rectal Activation Phenotype test could be used as a simple, highly sensitive, non-invasive biomarker to determine efficacy of dystrophin replacement therapies.

摘要

引言

杜氏肌营养不良症是一种由肌营养不良蛋白缺乏引起的遗传性疾病。杜氏肌营养不良症患者和患营养不良症的小鼠都存在肠道功能障碍。

方法

本研究源于在20分钟的强制连续跑步机运动期间偶然观察到患营养不良症的小鼠与正常小鼠粪便排出量的差异。在此，我们报告运动对两种不同的营养不良突变体及其正常背景对照品系粪便排出量的影响。对运动期间排出的所有粪便进行计数、干燥和称重。

结果

两种突变型营养不良品系的小鼠在运动期间产生的粪便量均显著多于相应的对照品系。

讨论

我们提出运动诱导的结肠 - 直肠激活表型测试可作为一种简单、高度敏感、非侵入性的生物标志物，用于确定肌营养不良蛋白替代疗法的疗效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1924/5959732/649f42f7c823/Figure-4-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1924/5959732/d4a5561800db/Figure-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1924/5959732/2d12e460db56/Figure-2-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1924/5959732/a62510e4eb1d/Figure-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1924/5959732/649f42f7c823/Figure-4-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1924/5959732/d4a5561800db/Figure-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1924/5959732/2d12e460db56/Figure-2-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1924/5959732/a62510e4eb1d/Figure-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1924/5959732/649f42f7c823/Figure-4-1.jpg

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本文引用的文献

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Current Translational Research and Murine Models For Duchenne Muscular Dystrophy.当前杜氏肌营养不良症的转化研究和小鼠模型。
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Constipation in Duchenne Muscular Dystrophy: Prevalence, Diagnosis, and Treatment.杜氏肌营养不良症中的便秘：患病率、诊断和治疗。
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Nutrition Considerations in Duchenne Muscular Dystrophy.杜氏肌营养不良症的营养考量
Nutr Clin Pract. 2015 Aug;30(4):511-21. doi: 10.1177/0884533615586202. Epub 2015 May 14.
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Intestine of dystrophic mice presents enhanced contractile resistance to stretching despite morphological impairment.营养不良的老鼠肠道在形态损伤的情况下，对拉伸的收缩阻力增强。
Am J Physiol Gastrointest Liver Physiol. 2014 Feb;306(3):G191-9. doi: 10.1152/ajpgi.00314.2013. Epub 2013 Nov 27.
5
Mutation types and aging differently affect revertant fiber expansion in dystrophic mdx and mdx52 mice.突变类型和衰老以不同的方式影响营养不良型 mdx 和 mdx52 小鼠的回复纤维扩张。
PLoS One. 2013 Jul 24;8(7):e69194. doi: 10.1371/journal.pone.0069194. Print 2013.
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Gastric emptying, small intestinal transit and fecal output in dystrophic (mdx) mice.营养不良（mdx）小鼠的胃排空、小肠转运和粪便排出。
J Physiol Sci. 2010 Jan;60(1):75-9. doi: 10.1007/s12576-009-0060-8. Epub 2009 Sep 26.
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Preclinical drug trials in the mdx mouse: assessment of reliable and sensitive outcome measures.杜兴氏肌营养不良症小鼠的临床前药物试验：可靠且敏感的结果指标评估
Muscle Nerve. 2009 May;39(5):591-602. doi: 10.1002/mus.21211.
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Feeding problems and weight gain in Duchenne muscular dystrophy.杜氏肌营养不良症中的喂养问题和体重增加
Eur J Paediatr Neurol. 2006 Sep-Nov;10(5-6):231-6. doi: 10.1016/j.ejpn.2006.08.008. Epub 2006 Oct 10.
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Evolution of gastric electrical features and gastric emptying in children with Duchenne and Becker muscular dystrophy.杜兴氏和贝克氏肌营养不良症患儿胃电特征及胃排空的演变
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