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肌营养不良蛋白在小鼠肌间神经元中的表达。

Expression of dystrophin in the mouse myenteric neurones.

作者信息

Vannucchi M G, Corsani L, Giovannini M G, Faussone-Pellegrini M S

机构信息

Department of Human Anatomy, Histology and Forensic Medicine, University of Florence, Italy.

出版信息

Neurosci Lett. 2001 Mar 9;300(2):120-4. doi: 10.1016/s0304-3940(01)01555-5.

Abstract

Dystrophin, a membrane-associated protein, plays relevant roles in cell functions. Its lack or trunkated expression results in Duchenne muscular dystrophy (DMD), a pathology associated with alterations in gastrointestinal motility considered to be neural in origin. No data are available on the presence of dystrophin in myenteric neurones. We labelled mouse myenteric neurones with DYS1-, DYS2-, DYS3-antibodies; staining was located on the perikarya and processes, with no differences in distribution or intensity among the antibodies; the western immunoblot analysis indicated that myenteric neurones express several dystrophin isoforms; anti-dystrophins/anti-neuronal specific enolase double-labeling confirmed that all neurones express dystrophin. Dystrophin in myenteric neurones might play a role in cytoskeletal organization, axonal transport and signal pathways; its lack might cause the intestinal motor abnormalities reported in DMD patients.

摘要

肌营养不良蛋白是一种与膜相关的蛋白质,在细胞功能中发挥着重要作用。其缺失或截短表达会导致杜氏肌营养不良症(DMD),这是一种与胃肠动力改变相关的病理状况,被认为起源于神经。关于肌间神经丛神经元中肌营养不良蛋白的存在情况尚无数据。我们用DYS1、DYS2、DYS3抗体标记小鼠肌间神经丛神经元;染色位于胞体和突起上,各抗体之间在分布或强度上无差异;蛋白质免疫印迹分析表明,肌间神经丛神经元表达多种肌营养不良蛋白亚型;抗肌营养不良蛋白/抗神经元特异性烯醇化酶双重标记证实所有神经元均表达肌营养不良蛋白。肌间神经丛神经元中的肌营养不良蛋白可能在细胞骨架组织、轴突运输和信号通路中发挥作用;其缺失可能导致DMD患者出现肠道运动异常。

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