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福山型先天性肌营养不良症肌细胞膜中正交阵列的小尺寸

Small size of orthogonal array in muscle plasma membrane of Fukuyama type congenital muscular dystrophy.

作者信息

Wakayama Y, Kumagai T, Jimi T

出版信息

Acta Neuropathol. 1986;72(2):130-3. doi: 10.1007/BF00685974.

Abstract

Freeze fracture analysis was carried out on the density of orthogonal array subunit particles in the plasma membrane of skeletal muscle of six patients with Fukuyama type congenital muscular dystrophy and seven control cases. The group mean density of orthogonal array subunit particles per one orthogonal array was significantly lower in the plasma membrane of Fukuyama patients. The results suggested the possible impairment of orthogonal array function in the plasma membrane of muscle fibers in congenital muscular dystrophy of the Fukuyama type.

摘要

对6例福山型先天性肌营养不良患者和7例对照者的骨骼肌质膜中正交阵列亚基颗粒的密度进行了冷冻断裂分析。福山型患者质膜中每个正交阵列的正交阵列亚基颗粒的组平均密度显著降低。结果提示福山型先天性肌营养不良患者肌纤维质膜中正交阵列功能可能受损。

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