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杜兴氏肌营养不良症:体外单纤维的异常激活。

Duchenne muscular dystrophy: unusual activation of single fibers in vitro.

作者信息

Takagi A, Nonaka I

出版信息

Muscle Nerve. 1981 Jan-Feb;4(1):10-5. doi: 10.1002/mus.880040104.

DOI:10.1002/mus.880040104
PMID:7231441
Abstract

Single skinned fibers were prepared from muscle biopsies of patients with Duchenne muscular dystrophy (DMD) and other neuromuscular diseases. The activation of the contractile system by calcium or strontium ion and the function of the sarcoplasmic reticulum were studied. In many fibers of the biopsy specimens from patients with DMD, activation by strontium revealed a pattern intermediate between those shown by the usual type 1 and type 2 fibers. These fibers were generally of smaller diameter and produced less maximal tension than type 1 and 2 fibers. The study of muscle of newborn animals suggests that this unusual pattern of activation might reflect either arrested development or incomplete regeneration after muscle necrosis in the disease.

摘要

从杜兴氏肌营养不良症(DMD)患者和其他神经肌肉疾病患者的肌肉活检样本中制备单根肌纤维。研究了钙离子或锶离子对收缩系统的激活作用以及肌浆网的功能。在DMD患者活检样本的许多肌纤维中,锶离子激活呈现出介于常见的1型和2型纤维之间的一种模式。这些纤维的直径通常较小,产生的最大张力也比1型和2型纤维小。对新生动物肌肉的研究表明,这种异常的激活模式可能反映了疾病中肌肉坏死后发育停滞或再生不完全的情况。

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1
Duchenne muscular dystrophy: unusual activation of single fibers in vitro.杜兴氏肌营养不良症:体外单纤维的异常激活。
Muscle Nerve. 1981 Jan-Feb;4(1):10-5. doi: 10.1002/mus.880040104.
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引用本文的文献

1
Contractile properties of muscles in myotonic dystrophy.强直性肌营养不良症中肌肉的收缩特性。
J Neurol Neurosurg Psychiatry. 1983 Jul;46(7):625-31. doi: 10.1136/jnnp.46.7.625.
2
Demonstration of cathepsins B, H and L in xenografts of normal and Duchenne-muscular-dystrophy muscles transplanted into nude mice.正常肌肉和杜兴氏肌营养不良症肌肉移植到裸鼠体内的异种移植中组织蛋白酶B、H和L的显示
Biochem J. 1992 Dec 1;288 ( Pt 2)(Pt 2):643-8. doi: 10.1042/bj2880643.