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金毛寻回犬肌肉萎缩症中腓骨长肌的收缩张力和动力学

Contraction tension and kinetics of the peroneus longus muscle in golden retriever muscular dystrophy.

作者信息

Kornegay J N, Sharp N J, Bogan D J, Van Camp S D, Metcalf J R, Schueler R O

机构信息

Companion Animal and Special Species Medicine, College of Veterinary Medicine, North Carolina State University, Raleigh 27606.

出版信息

J Neurol Sci. 1994 May;123(1-2):100-7. doi: 10.1016/0022-510x(94)90210-0.

Abstract

Contraction tension and kinetics of the peroneus longus muscle were studied in dogs with the Duchenne homologue, golden retriever muscular dystrophy (GRMD), in advance of evaluating localized therapies such as myoblast transplantation. Absolute and both muscle- and body-weight-corrected twitch tension in GRMD dogs were low compared to normal litter mates at 3 months of age (p < 0.0005 for all). Tetanic tension was affected similarly. However, whereas absolute values were still reduced at 6 months (p < 0.0005 for twitch and 0.005 for tetany), twitch and tetanic tension corrected for either muscle or body weight was not statistically different, suggesting that the peroneus longus may be relatively spared in GRMD. Post-tetanic potentiation was more pronounced in GRMD versus normal dogs at both 3 (p < 0.0001) and 6 (p < 0.01) months. The degree of positive staircase at 3 months of age did not differ. Twitch contraction and relaxation times were dramatically prolonged, and there was concomitant sustained electrical activity, at, or before, 6 months of age in some severely affected dogs. Relatively few carriers were evaluated at these ages, but their values were similar to those of normal dogs. Apparent sparing of the peroneus longus muscle may limit application of this technique to evaluation of therapies administered early in life or in combination with toxins. Treatment to alter changes in contraction kinetics could also be assessed.

摘要

在评估成肌细胞移植等局部治疗方法之前,对患有杜兴氏同源疾病——金毛寻回犬型肌营养不良症(GRMD)的犬只的腓骨长肌收缩张力和动力学进行了研究。与3个月大的正常同窝仔犬相比,GRMD犬的绝对以及经肌肉和体重校正后的抽搐张力均较低(所有比较的p值均<0.0005)。强直张力也受到类似影响。然而,虽然6个月大时绝对值仍降低(抽搐的p值<0.0005,强直的p值<0.005),但经肌肉或体重校正后的抽搐和强直张力在统计学上并无差异,这表明GRMD中腓骨长肌可能相对未受影响。在3个月(p<0.0001)和6个月(p<0.01)时,GRMD犬的强直后增强比正常犬更明显。3个月大时的正阶梯现象程度无差异。在6个月龄时或之前,一些严重受影响的犬只的抽搐收缩和舒张时间显著延长,同时伴有持续的电活动。在这些年龄段评估的携带者相对较少,但他们的值与正常犬相似。腓骨长肌明显未受影响可能会限制该技术在评估生命早期给予的治疗或与毒素联合使用的治疗中的应用。也可以评估改变收缩动力学变化的治疗方法。

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