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首页> 外文期刊>Brain pathology >Neuropathological staging of spinocerebellar ataxia type 2 by semiquantitative 1c2-positive neuron typing. Nuclear translocation of cytoplasmic 1C2 underlies disease progression of spinocerebellar ataxia type 2
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Neuropathological staging of spinocerebellar ataxia type 2 by semiquantitative 1c2-positive neuron typing. Nuclear translocation of cytoplasmic 1C2 underlies disease progression of spinocerebellar ataxia type 2

机译：半定量1c2阳性神经元分型对2型脊髓小脑共济失调的神经病理分期。细胞质1C2的核易位是2型脊髓小脑共济失调疾病进展的基础

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Spinocerebellar ataxia type 2 (SCA2) is a hereditary neurodegenerative disorder caused by the expansion of the trinucleotide CAG repeats encoding elongated polyglutamine tract in ataxin-2, the SCA2 gene product. Polyglutamine diseases comprise nine genetic entities, including seven different forms of spinocerebellar ataxias, Huntington's disease, and spinal and bulbar muscular atrophy. These are pathologically characterized by neuronal loss and intranuclear aggregates or inclusions of mutant proteins including expanded polyglutamine in selected neuronal groups. Previously, we examined immunolocalization of ubiquitin, expanded polyglutamine (probed by 1C2 antibody), and ataxin-2 in genetically confirmed SCA2 patients. In the present study, we expanded this approach by distinguishing different patterns of subcellular 1C2 immunoreactivity ("granular cytoplasmic," "cytoplasmic and nuclear" and "nuclear with inclusions.") and by quantifying their regional frequencies in three autopsied SCA2 brains at different stage of the disease. Comparison with neuronal loss and gliosis revealed that overall 1C2 immunoreactivity was paralleled with their severity. Furthermore, appearance of granular cytoplasmic pattern corresponded to early stage, cytoplasmic and nuclear pattern to active stage, and nuclear with inclusions pattern to final stage. We conclude that this 1C2-immunoreactive typing may be useful for evaluating the overall severity and extent of affected regions and estimating the neuropathological stage of SCA2.
机译：脊髓小脑性共济失调2型（SCA2）是一种遗传性神经退行性疾病，由SCA2基因产物ataxin-2中编码细长聚谷氨酰胺束的三核苷酸CAG重复序列的扩增引起。聚谷氨酰胺疾病包括九种遗传实体，包括七种不同形式的脊髓小脑共济失调，亨廷顿舞蹈病以及脊髓和延髓性肌萎缩。这些在病理学上的特征是神经元丢失和核内聚集或包含突变蛋白的包涵体，包括选定神经元组中的聚谷氨酰胺。以前，我们在基因确认的SCA2患者中检查了泛素，扩展的聚谷氨酰胺（由1C2抗体探测）和紫杉醇2的免疫定位。在本研究中，我们通过区分亚细胞1C2免疫反应性的不同模式（“颗粒细胞质”，“细胞质和核”和“带有包涵体的核”）并通过量化在三个不同阶段的三个解剖SCA2脑中的区域频率来扩展这种方法。这种疾病。与神经元丢失和神经胶质增生的比较显示，总体1C2免疫反应性与其严重程度平行。此外，粒状胞质模式的出现对应于早期，胞质和核模式对应于活跃期，核内含物模式对应于最终阶段。我们得出的结论是，这种1C2免疫反应分型可能对评估受影响区域的总体严重性和程度以及评估SCA2的神经病理学阶段有用。

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