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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Distinctive features of degenerating Purkinje cells in spinocerebellar ataxia type 31
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Distinctive features of degenerating Purkinje cells in spinocerebellar ataxia type 31

机译：脊髓小脑共济失调31型变性浦肯野细胞的特征

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摘要

Spinocerebellar ataxia type 31 (SCA31) is an autosomal dominant form of pure cerebellar ataxia that is caused by a disease-specific insertion containing penta-nucleotide repeats (TGGAA)n. Neuropathologically, cerebellar Purkinje cells are preferentially affected and reduced in number in SCA31, and they are often surrounded by halo-like amorphous materials. In the present study, we performed neuropathological analyses on two SCA31 brains, and discussed the serial morphological changes of Purkinje cells in SCA31.We found that bent, elongated, often folded nuclei were observed frequently in degenerating Purkinje cells with the halo-like structure. Conversely, Purkinje cells without this structure developed marked atrophy with severely slender and condensed nuclei. On the basis of these pathological findings, we propose two different processes for Purkinje cell degeneration in SCA31, namely, shrinkage of Purkinje cells with or without the halo-like amorphous materials. The former, but not the latter, was considered to be specific to SCA31. Correspondingly, fragmentation of the Golgi apparatus was observed more frequently in Purkinje cells with the halo-like structure than in those without this structure. We consider that the profound nuclear deformity and fragmentation of the Golgi apparatus are closely linked with the formation of the halo-like structure in SCA31.
机译：脊髓小脑性共济失调类型31（SCA31）是纯小脑性共济失调的常染色体显性形式，由疾病特异性插入包含五核苷酸重复序列（TGGAA）n引起。在神经病理学上，小脑浦肯野细胞在SCA31中受到优先影响并数量减少，并且通常被类似晕轮状的无定形物质所包围。在本研究中，我们对两个SCA31大脑进行了神经病理学分析，并讨论了SCA31中Purkinje细胞的系列形态变化，我们发现在退化具有晕轮状结构的Purkinje细胞中经常观察到弯曲，细长，经常折叠的核。相反，不具有这种结构的浦肯野细胞则出现明显的萎缩，细胞核非常细长且密集。基于这些病理学发现，我们提出了SCA31中浦肯野细胞变性的两种不同过程，即有或没有晕轮状无定形物质的浦肯野细胞的收缩。前者（而非后者）被认为特定于SCA31。相应地，在具有晕轮状结构的浦肯野细胞中比在没有这种结构的那些中更频繁地观察到高尔基体的破碎。我们认为高尔基体的深部核畸变和碎片化与SCA31中类晕结构的形成紧密相关。

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