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Axonal changes in experimental prion diseases recapitulate those following constriction of postganglionic branches of the superior cervical ganglion: a comparison 40 years later.
Prion ( IF 1.9 ) Pub Date : 2019-04-09 , DOI: 10.1080/19336896.2019.1595315
Paweł P Liberski 1
Affiliation  

The major neurological feature of prion diseases is a neuronal loss accomplished through either apoptosis or autophagy. In this review, I compared axonal alterations in prion diseases to those described 40 years earlier as a result of nerve ligation. I also demonstrated that autophagic vacuoles and autophagosomes are a major part of dystrophic neurites. Furthermore, I summarized the current status of the autophagy in prion diseases and hypothesize, that spongiform change may originate from the autophagic vacuoles. This conclusion should be supported by other methods, in particular laser confocal microscopy.

We observed neuronal autophagic vacuoles in different stages of formation, and our interpretation of the ‘maturity’ of their formation may or may not equate to actual developmental stages. Initially, a part of the neuronal cytoplasm was sequestrated within double or multiple membranes (phagophores) and often exhibited increased electron-density. The intracytoplasmic membranes formed labyrinth-like structures that suggest a multiplication of those membranes. The autophagic vacuoles then expand and eventually, a vast area of the cytoplasm was transformed into a merging mass of autophagic vacuoles. Margaret R. Matthews published a long treatise in the Philosophical Transactions of the Royal Society of London in which she had described in great detail the ultrastructure of postganglionic branches of the superior cervical ganglion in the rat following ligation of them. The earliest changes observed by Matthews between 6 h to 2 days in the proximal stump were distensions of proximal axons. Analogously, in our models, an increased number of ‘regular’ (round) and ‘irregular’ MVB and some autophagic vacuoles were observed collectively, both processes were similar.



中文翻译:

实验性pr病毒疾病的轴突变化概括了上颈神经节的神经节后分支收缩后的情况:40年后的比较。

ion病毒疾病的主要神经系统特征是通过凋亡或自噬实现的神经元丧失。在这篇综述中,我将40病毒疾病的轴突改变与40年前由于神经结扎而描述的改变进行了比较。我还证明自噬空泡和自噬小体是营养不良性神经突的主要部分。此外,我总结了病毒疾病中自噬的现状,并假设海绵状变化可能源于自噬泡。该结论应得到其他方法的支持,尤其是激光共聚焦显微镜。

我们观察到神经元自噬空泡在形成的不同阶段,我们对其形成的“成熟度”的解释可能等于或可能不等于实际的发育阶段。最初,一部分神经元细胞质被隔离在双层或多层膜(吞噬细胞)中,并经常表现出更高的电子密度。胞浆内膜形成迷宫状结构,表明这些膜的繁殖。然后自噬泡扩展,最后,大范围的细胞质被转化为合并的自噬泡。玛格丽特·马修斯(Margaret R. Matthews)在伦敦皇家学会的哲学交易》上发表了一篇长篇论文。她详细描述了结扎大鼠上颈神经节后神经节后分支的超微结构。马修斯在近端残端6小时至2天之间观察到的最早变化是近端轴突膨胀。类似地,在我们的模型中,共同观察到越来越多的“规则”(圆形)和“不规则” MVB以及一些自噬泡,这两个过程是相似的。

更新日期:2019-04-09
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