肌营养不良蛋白和β-肌营养不良聚糖mRNA在人骨骼肌中肌膜下的优先定位。

Preferential subsarcolemmal localization of dystrophin and beta-dystroglycan mRNA in human skeletal muscles.

作者信息

Mitsui T, Kawai H, Shono M, Kawajiri M, Kunishige M, Saito S

机构信息

First Department of Internal Medicine, School of Medicine, The University of Tokushima, Kuramoto-3, Japan.

出版信息

J Neuropathol Exp Neurol. 1997 Jan;56(1):94-101. doi: 10.1097/00005072-199701000-00010.

DOI:10.1097/00005072-199701000-00010

PMID:8990133

Abstract

The intracellular localization of dystrophin and beta-dystroglycan mRNA in skeletal muscles of patients with Duchenne muscular dystrophy (DMD) or Becker muscular dystrophy (BMD) and normal subjects was examined by in situ hybridization using biotinylated oligonucleotide probes. These mRNAs were found preferentially in sarcolemma in the skeletal muscles of both normal subjects and affected patients. Quantitative analysis of mRNA signals demonstrated no prominent reduction of dystrophin or beta-dystroglycan mRNA in DMD/BMD muscles. These results suggest that even mRNAs with deletions contain specific information that affects their localization, and the characteristic defect of dystrophin in DMD/BMD muscles seems to be caused mainly by the instability of dystrophin protein, as a post-transcriptional event.

摘要

利用生物素化寡核苷酸探针，通过原位杂交技术检测了杜氏肌营养不良症（DMD）或贝克肌营养不良症（BMD）患者以及正常受试者骨骼肌中肌营养不良蛋白和β - 肌营养不良聚糖mRNA的细胞内定位。在正常受试者和患病患者的骨骼肌中，这些mRNA均优先定位于肌膜。对mRNA信号的定量分析表明，DMD/BMD肌肉中肌营养不良蛋白或β - 肌营养不良聚糖mRNA没有显著减少。这些结果表明，即使是有缺失的mRNA也包含影响其定位的特定信息，并且DMD/BMD肌肉中肌营养不良蛋白的特征性缺陷似乎主要是由转录后事件中肌营养不良蛋白的不稳定性引起的。