Structural study of Purkinje cell axonal torpedoes in essential tremor.
Structural study of Purkinje cell axonal torpedoes in essential tremor.
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DOI：
10.1016/j.neulet.2008.11.043
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发表时间：
2009-02-06
影响因子：
2.5
通讯作者：
Faust PL
中科院分区：
文献类型：
--
作者：
Louis ED;Yi H;Erickson-Davis C;Vonsattel JP;Faust PL
特发性震颤（ET）是最常见的神经系统疾病之一。现在对其神经病理学的基本理解正在形成。除了浦肯野细胞丢失，一个突出的发现是大量的鱼雷（浦肯野细胞轴突的圆形突起）。这种肿胀通常是由于细胞成分的错误积累。确定这些积累的基本性质是理解潜在疾病过程的重要一步。鱼雷，最近才确定的ET，尚未进行超微结构的特点。光学和电子显微镜被用来表征鱼雷在ET的结构成分。对来自4个前瞻性收集的ET脑的福尔马林固定的小脑皮质组织进行切片，并用单克隆磷酸化神经丝抗体（SMI-31，Covance，Emeryville，CA）进行免疫染色。使用来自3个ET脑的额外切片，使用电子显微镜评估鱼雷。磷酸化神经丝蛋白的免疫反应性显示，在每种情况下，鱼雷清晰的标记。鱼雷具有强烈的免疫反应性;在许多情况下，注意到两个或更多个鱼雷彼此非常接近。在电子显微镜下，鱼雷填充有随机排列的10-12 nm神经丝。线粒体和滑面内质网也很丰富，特别是在鱼雷的周边。我们证明了ET中的鱼雷代表了混乱的神经丝和其他细胞器的错误积累。尚不清楚这些累积发生在致病级联的何处（即，这些积累是主要事件还是次要/下游事件），这值得进一步研究。
Essential tremor (ET) is one of the most common neurological diseases. A basic understanding of its neuropathology is now emerging. Aside from Purkinje cell loss, a prominent finding is an abundance of torpedoes (rounded swellings of Purkinje cell axons). Such swellings often result from the mis-accumulation of cell constituents. Identifying the basic nature of these accumulations is an important step in understanding the underlying disease process. Torpedoes, only recently identified in ET, have not yet been characterized ultrastructurally. Light and electron microscopy were used to characterize the structural constituents of torpedoes in ET. Formalin-fixed cerebellar cortical tissue from 4 prospectively-collected ET brains was sectioned and immunostained with a monoclonal phosphorylated neurofilament antibody (SMI-31, Covance, Emeryville, CA). Using additional sections from 3 ET brains, torpedoes were assessed using electron microscopy. Immunoreactivity for phosphorylated neurofilament protein revealed clear labeling of torpedoes in each case. Torpedoes were strongly immunoreactive; in many instances, two or more torpedoes were noted in close proximity to one another. On electron microscopy, torpedoes were packed with randomly arranged 10–12 nm neurofilaments. Mitochondria and smooth endoplasmic reticulum were abundant as well, particularly at the periphery of the torpedo. We demonstrated that the torpedoes in ET represent the mis-accumulation of disorganized neurofilaments and other organelles. It is not known where in the pathogenic cascade these accumulations occur (i.e., whether these accumulations are the primary event or a secondary/downstream event) and this deserves further study.
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影响因子：
64.5
作者：
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通讯作者：
CLEVELAND, DW
影响因子：
9.9
作者：
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通讯作者：
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影响因子：
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作者：
Beaulieu, J M;Nguyen, M D;Julien, J P
通讯作者：
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DOI：
10.1097/00005072-199209000-00008
发表时间：
1992-09-01
影响因子：
3.2
作者：
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通讯作者：
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影响因子：
2.5
作者：
MA, WY;VACCAGALLOWAY, LL
通讯作者：
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