Walsh F S, Moore S E, Dickson J G
Institute of Neurology, Queen Square, London, UK.
J Neurol Neurosurg Psychiatry. 1988 Jan;51(1):136-8. doi: 10.1136/jnnp.51.1.136.
Muscle biopsies from a series of myotonic dystrophy patients were analysed for expression of the nerve regulated gene products neural cell adhesion molecule (N-CAM) and 5.1H11. All eight biopsy specimens tested strongly expressed N-CAM and 5.1H11 as assessed by indirect immunofluorescence analysis. These results can be compared with those of Renaud et al (Nature, 1986;319:678) that show apamin binding to be a good marker of myotonic dystrophy muscle membranes. We suggest that in myotonic dystrophy a number of nerve regulated membrane markers are precociously expressed by innervated myofibres and that these are likely to be secondary manifestations resulting from an unidentified primary defect.
对一系列强直性肌营养不良患者的肌肉活检样本进行分析,以检测神经调节基因产物神经细胞黏附分子(N-CAM)和5.1H11的表达情况。通过间接免疫荧光分析评估,所有检测的八份活检标本均强烈表达N-CAM和5.1H11。这些结果可与雷诺等人(《自然》,1986年;319:678)的结果相比较,他们的研究表明蜂毒明肽结合是强直性肌营养不良肌膜的一个良好标志物。我们认为,在强直性肌营养不良中,一些神经调节的膜标志物由受神经支配的肌纤维过早表达,并且这些可能是由未明确的原发性缺陷导致的继发性表现。