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[强直性肌营养不良患者肌肉病理学与DM激酶异常之间的关系]

[The relationship between muscle pathology and DM kinase abnormalities in patients with dystrophia myotonica].

作者信息

Murata K, Takayanagi T, Matsumura R, Murata K

机构信息

Department of Neurology Nara Medical University, Japan.

出版信息

Rinsho Shinkeigaku. 1996 Sep;36(9):1055-9.

PMID:8976127
Abstract

We studied the relationship between muscle pathology and the number of trinucleotide (CTG) repeats observed in lymphocytes and biopsied muscle tissues from patients with dystrophia myotonica (DM). The diameter of type 1 muscle fibers was smaller than that of type 2 fibers in all patients. The diameter and proportion of each muscle fiber type were related to the patient's age, but not to the number of trinucleotide (CTG) repeats of DM kinase in biopsied muscles. On the other hand, the proportion of type 1 fibers with central nuclei was closely related to the number of trinucleotide (CTG) repeats in muscles. These findings suggest that DM kinase abnormalities influence the muscle nuclei, and increase the number of central nuclei.

摘要

我们研究了强直性肌营养不良症(DM)患者淋巴细胞和肌肉活检组织中观察到的肌肉病理学与三核苷酸(CTG)重复序列数量之间的关系。所有患者中1型肌纤维的直径均小于2型肌纤维。每种肌纤维类型的直径和比例与患者年龄有关,但与活检肌肉中DM激酶的三核苷酸(CTG)重复序列数量无关。另一方面,有中央核的1型纤维比例与肌肉中三核苷酸(CTG)重复序列数量密切相关。这些发现表明,DM激酶异常会影响肌细胞核,并增加中央核的数量。

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