Bora Gamze, Subaşı-Yıldız Şulenur, Yeşbek-Kaymaz Ayşe, Bulut Numan, Alemdaroğlu İpek, Tunca-Yılmaz Öznur, Topaloğlu Haluk, Karaduman Aynur Ayşe, Erdem-Yurter Hayat
1 Faculty of Medicine, Department of Medical Biology, Hacettepe University, Ankara, Turkey.
2 Faculty of Health Sciences, Department of Physical Therapy and Rehabilitation, Hacettepe University, Ankara, Turkey.
J Child Neurol. 2018 Mar;33(3):209-215. doi: 10.1177/0883073817750500. Epub 2018 Jan 12.
Exercise studies in neuromuscular diseases like spinal muscular atrophy (SMA), a devastating disease caused by survival of motor neuron 1 ( SMN1) gene mutations, are drawing attention due to its beneficial effects. In this study, we presented a constructed arm cycling exercise protocol and evaluated the benefits on SMA patients. Five SMA type II patients performed 12 weeks of supervised arm cycling exercise. The physical functions were evaluated together with the SMN2 copy numbers, SMN protein levels, insulin-like growth factor 1(IGF1) and binding protein 3 (IGFBP3) levels. The active cycling distance and duration of patients significantly improved. Significant changes could not have detected either SMN or IGF1 and IGFBP3 levels in response to exercise. The findings demonstrated that the patients tolerated the exercise protocol and gained a benefit from arm cycling but benefits could not be associated with SMN2 copy number, SMN protein level, IGF1, or IGFBP3 levels.
针对诸如脊髓性肌萎缩症（SMA）这类神经肌肉疾病的运动研究正受到关注，因为其具有有益效果。脊髓性肌萎缩症是一种由运动神经元存活1（SMN1）基因突变导致的毁灭性疾病。在本研究中，我们提出了一种构建的手臂循环运动方案，并评估了其对SMA患者的益处。五名II型SMA患者进行了为期12周的有监督的手臂循环运动。对身体功能进行了评估，并同时检测了SMN2拷贝数、SMN蛋白水平、胰岛素样生长因子1（IGF1）和结合蛋白3（IGFBP3）水平。患者的主动骑行距离和时长显著改善。运动后未检测到SMN、IGF1和IGFBP3水平有显著变化。研究结果表明，患者耐受该运动方案并从手臂循环运动中获益，但这种益处与SMN2拷贝数、SMN蛋白水平、IGF1或IGFBP3水平无关。
