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运动对亨廷顿病骨骼肌病理生理学的影响

Effects of Exercise on Skeletal Muscle Pathophysiology in Huntington's Disease.

作者信息

Trovato Bruno, Magrì Benedetta, Castorina Alessandro, Maugeri Grazia, D'Agata Velia, Musumeci Giuseppe

机构信息

Department of Biomedical and Biotechnological Sciences, Human, Histology and Movement Science Section, University of Catania, Via S. Sofia n°87, 95123 Catania, Italy.

Laboratory of Cellular and Molecular Neuroscience (LCMN), School of Life Sciences, Faculty of Science, University of Technology Sydney, Sydney, NSW 2007, Australia.

出版信息

J Funct Morphol Kinesiol. 2022 May 11;7(2):40. doi: 10.3390/jfmk7020040.

Abstract

Huntington's disease (HD) is a rare, hereditary, and progressive neurodegenerative disease, characterized by involuntary choreatic movements with cognitive and behavioral disturbances. In order to mitigate impairments in motor function, physical exercise was integrated in HD rehabilitative interventions, showing to be a powerful tool to ameliorate the quality of life of HD-affected patients. This review aims to describe the effects of physical exercise on HD-related skeletal muscle disorders in both murine and human models. We performed a literature search using PubMed, Scopus, and Web of Science databases on the role of physical activity in mouse models of HD and human patients. Fifteen publications fulfilled the criteria and were included in the review. Studies performed on mouse models showed a controversial role played by exercise, whereas in HD-affected patients, physical activity appeared to have positive effects on gait, motor function, UHDMRS scale, cognitive function, quality of life, postural stability, total body mass, fatty acid oxidative capacity, and VO2 max. Physical activity seems to be feasible, safe, and effective for HD patients. However, further studies with longer follow-up and larger cohorts of patients will be needed to draw firm conclusions on the positive effects of exercise for HD patients.

摘要

亨廷顿病(HD)是一种罕见的、遗传性的进行性神经退行性疾病,其特征为伴有认知和行为障碍的不自主舞蹈样运动。为减轻运动功能障碍,体育锻炼被纳入HD康复干预措施中,显示出是改善HD患者生活质量的有力工具。本综述旨在描述体育锻炼对小鼠和人类模型中与HD相关的骨骼肌疾病的影响。我们使用PubMed、Scopus和Web of Science数据库对体育活动在HD小鼠模型和人类患者中的作用进行了文献检索。15篇出版物符合标准并被纳入综述。对小鼠模型进行的研究显示锻炼所起的作用存在争议,而在HD患者中,体育活动似乎对步态、运动功能、统一亨廷顿病评定量表(UHDMRS)评分、认知功能、生活质量、姿势稳定性、总体重、脂肪酸氧化能力和最大摄氧量(VO2 max)有积极影响。体育活动对HD患者似乎是可行、安全且有效的。然而,需要进行更长随访时间和更大患者队列的进一步研究,以就锻炼对HD患者的积极影响得出确凿结论。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8e1e/9149967/5c211052ec70/jfmk-07-00040-g001.jpg

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