发育中的视网膜中的肌营养不良蛋白：Dp260表达与突触成熟相关。

Dystrophins in developing retina: Dp260 expression correlates with synaptic maturation.

作者信息

Rodius F, Claudepierre T, Rosas-Vargas H, Cisneros B, Montanez C, Dreyfus H, Mornet D, Rendon A

机构信息

INSERM CJF 92/02, Laboratoire de Physiopathologie Rétinienne, Université Louis Pasteur, CHRU, Strasbourg, France.

出版信息

Neuroreport. 1997 Jul 7;8(9-10):2383-7. doi: 10.1097/00001756-199707070-00056.

DOI:10.1097/00001756-199707070-00056

PMID:9243645

Abstract

Dystrophin, the protein altered in Duchenne muscular dystrophy (DMD), is necessary for normal retinal function and exists in several isoforms. We examined the expression of dystrophin and utrophin proteins and transcripts in the rat retina at different developmental stages using Western blots and semi-quantitative RT-PCR. Our results revealed the presence of utrophin (DRP1), G-utrophin and/or DRP2 and four dystrophin isoforms (Dp427, Dp260, Dp140, Dp71) in the normal adult rat retina. Only Dp260 showed a marked progressive increase with age at both protein and mRNA levels. This variation is consistent with the establishment of synaptic functions in the developing retina and suggests a key role for this apo-dystrophin in synaptogenesis.

摘要

肌营养不良蛋白是杜氏肌营养不良症（DMD）中发生改变的蛋白质，对正常视网膜功能至关重要，且存在多种同工型。我们使用蛋白质免疫印迹法和半定量逆转录聚合酶链反应（RT-PCR）检测了不同发育阶段大鼠视网膜中肌营养不良蛋白和抗肌萎缩蛋白的蛋白质及转录本表达情况。我们的结果显示，正常成年大鼠视网膜中存在抗肌萎缩蛋白（DRP1）、G-抗肌萎缩蛋白和/或DRP2以及四种肌营养不良蛋白同工型（Dp427、Dp260、Dp140、Dp71）。只有Dp26 在蛋白质和mRNA水平上均随年龄增长呈现显著的渐进性增加。这种变化与发育中视网膜突触功能的建立相一致，表明这种脱辅基肌营养不良蛋白在突触形成中起关键作用。