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对坐骨神经中肌营养不良蛋白和抗肌萎缩蛋白分布的重新评估。

Re-evaluation of the distributions of dystrophin and utrophin in sciatic nerve.

作者信息

Fabbrizio E, Latouche J, Rivier F, Hugon G, Mornet D

机构信息

INSERM U 300, Faculté de Pharmacie, Montpellier, France.

出版信息

Biochem J. 1995 Nov 15;312 ( Pt 1)(Pt 1):309-14. doi: 10.1042/bj3120309.

Abstract

Differential expression of proteins belonging to the dystrophin family was analysed in peripheral nerves. In agreement with previous reports, no full-size dystrophin was detectable, only Dp116, one of the short dystrophin products of the Duchenne muscular dystrophy (DMD) gene. We used specific monoclonal antibodies to fully investigate the presence of utrophin, a dystrophin homologue encoded by a gene located on chromosome 6q24. Evidence is presented here of the presence of two potential isoforms of full-length utrophin in different nerve structures, which may differ by alternative splicing of the 3'-terminal part of the utrophin gene according to the specificities of the monoclonal antiobodies used. One full-length utrophin was co-localized with Dp116 in the sheath around each separate Schwann cell-axon unit, but the other utrophin isoform was found to be perineurium-specific. We also highlighted a potential 80 kDa utrophin-related protein. The utrophin distribution in peripheral nerves was re-evaluated and utrophin isoforms were detected at the protein level. This preliminary indication will require more concrete molecular evidence to confirm the presence of these two utrophin isoforms as well as the potential 80 kDa utrophin isoform, but the results strongly suggest that each isoform must have a specialized role and function within each specific nervous structure.

摘要

对周围神经中肌营养不良蛋白家族相关蛋白的差异表达进行了分析。与先前的报道一致,未检测到全长肌营养不良蛋白,仅检测到杜氏肌营养不良症(DMD)基因的短肌营养不良蛋白产物之一Dp116。我们使用特异性单克隆抗体全面研究了抗肌萎缩蛋白聚糖的存在情况,抗肌萎缩蛋白聚糖是一种由位于6号染色体q24上的基因编码的肌营养不良蛋白同源物。本文提供的证据表明,在不同神经结构中存在两种潜在的全长抗肌萎缩蛋白聚糖同工型,根据所使用单克隆抗体的特异性,它们可能因抗肌萎缩蛋白聚糖基因3'-末端部分的可变剪接而有所不同。一种全长抗肌萎缩蛋白聚糖与Dp116共定位于每个单独的施万细胞-轴突单元周围的鞘中,但另一种抗肌萎缩蛋白聚糖同工型被发现是神经束膜特异性的。我们还发现了一种潜在的80 kDa抗肌萎缩蛋白聚糖相关蛋白。重新评估了抗肌萎缩蛋白聚糖在周围神经中的分布,并在蛋白质水平检测到了抗肌萎缩蛋白聚糖同工型。这一初步迹象需要更多具体的分子证据来证实这两种抗肌萎缩蛋白聚糖同工型以及潜在的80 kDa抗肌萎缩蛋白聚糖同工型的存在,但结果强烈表明,每种同工型在每个特定神经结构中必定具有专门的作用和功能。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a31/1136260/7d7d9b3f36dd/biochemj00051-0301-a.jpg

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