Long-term, age-associated activity quantification in the DE50-MD dog model of Duchenne muscular dystrophy.

IF 3.3 3区 医学 Q2 CELL BIOLOGY
Disease Models & Mechanisms Pub Date : 2025-07-01 Epub Date: 2025-07-14 DOI:10.1242/dmm.052135
Kamila Karimjee, Rachel C M River, Emil Olsen, Yu-Mei Chang, Dominic J Wells, Monica A Daley, Richard J Piercy
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引用次数: 0

Abstract

Animal models with a clinically relevant phenotype remain important for robust evaluation of novel therapeutics for the fatal X-linked genetic disorder Duchenne muscular dystrophy (DMD). Demonstration of functional improvement is crucial for both patients and regulatory authorities. Here, we investigate non-invasive methods to quantify activity changes in DE50-MD dogs. Using collar-based Axivity-AX3 tri-axial accelerometers, we measured activity in affected DE50-MD male dogs (3-8 per age point) and littermate wild-type (WT) male controls (3-13 per age point) at monthly intervals from 3 to 18 months of age. Acceleration vector magnitudes were used to derive a series of activity measures over 24 h. Mixed model analyses were used to examine differences between affected and WT groups at different ages. Activity indicators for DE50-MD dogs were significantly higher for percent time spent at rest (P<0.001) and significantly lower for all other activity indicators (all P<0.05), when compared to age-matched WT dogs. Relatively few animals would be required to detect treatment effects with adequate power using these unbiassed, selected and composite activity measures. Our approach reveals opportunities for cross-model standardisation of activity monitoring.

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杜氏肌营养不良(DMD) DE50-MD犬模型的长期、年龄相关活动量化。
具有临床相关表型的动物模型对于评估致命的x连锁遗传性疾病杜氏肌营养不良症(DMD)的新疗法仍然很重要。功能改善的证明对患者和监管当局都至关重要。在这里,我们研究了非侵入性方法来量化DE50-MD犬的活动变化,使用基于项圈的三轴加速度计。使用Axivity-AX3加速计,我们每月测量受影响的DE50-MD雄性狗(每个年龄点3-8只)和同窝野生型(WT)雄性对照狗(每个年龄点3-13只)的活动,时间间隔为3至18个月。加速度矢量震级用于推导24小时内的一系列活动测量。混合模型分析用于检验不同年龄的受影响组和WT组之间的差异。DE50-MD犬的活动指标明显高于休息时间(p
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来源期刊
Disease Models & Mechanisms
Disease Models & Mechanisms 医学-病理学
CiteScore
6.60
自引率
7.00%
发文量
203
审稿时长
6-12 weeks
期刊介绍: Disease Models & Mechanisms (DMM) is an online Open Access journal focusing on the use of model systems to better understand, diagnose and treat human disease.
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