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杜兴氏肌营养不良症单根肌纤维中的肌球蛋白轻链成分

Myosin light chain components in single muscle fibers of Duchenne muscular dystrophy.

作者信息

Takagi A, Ishiura S, Nonaka I, Sugita H

出版信息

Muscle Nerve. 1982 May-Jun;5(5):399-404. doi: 10.1002/mus.880050511.

Abstract

Single muscle fibers were prepared from biopsy specimens of Duchenne muscular dystrophy (DMD), normal, and neuromuscular disease controls. Single muscle cells were classified as type 1, type 2, or intermediate by the skinned fiber method. The intermediate fiber was most abundant in DMD, comprising 29% of fibers examined. The fiber type of single muscle fibers was contrasted to the composition of myosin light chain (MLC) components, which was analyzed by micro two-dimensional gel electrophoresis. In DMD, each of the components exhibited the same electrophoretic mobility as those in the controls. Type 1 fibers of DMD were more diverse in the composition of MLC than those of controls; 55% of type 1 fibers of DMD contained distinct fast-type MLC 3. Some intermediate fibers contained all five MLC components, but in others the composition was not different from usual type 1 or type 2 fibers. The diversity of MLC composition in DMD muscle cells might reflect the abundance of young muscle fibers in the tissue due to active muscle regeneration and/or retardation of maturation.

摘要

从杜兴氏肌营养不良症(DMD)患者、正常人和神经肌肉疾病对照组的活检标本中制备单根肌纤维。采用皮肤纤维法将单根肌细胞分为1型、2型或中间型。中间型纤维在DMD中最为丰富,占所检查纤维的29%。将单根肌纤维的纤维类型与通过微量二维凝胶电泳分析的肌球蛋白轻链(MLC)成分组成进行对比。在DMD中,每种成分的电泳迁移率与对照组相同。DMD的1型纤维在MLC组成上比对照组更多样化;DMD的1型纤维中有55%含有独特的快肌型MLC 3。一些中间型纤维含有所有五种MLC成分,但在其他纤维中,其组成与通常的1型或2型纤维没有差异。DMD肌细胞中MLC组成的多样性可能反映了由于活跃的肌肉再生和/或成熟延迟,组织中年轻肌纤维的丰富。

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