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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Accumulation of phosphorylated TDP-43 in the CNS of a patient with Cockayne syndrome.
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Accumulation of phosphorylated TDP-43 in the CNS of a patient with Cockayne syndrome.

机译：患有Cockayne综合征的患者中枢神经系统中磷酸化TDP-43的积累。

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摘要

Here, we report a case of Cockayne syndrome (CS) in a Japanese man who displayed a unique pathology of phosphorylated trans-activation response (TAR) DNA-binding protein 43 (pTDP-43) with abundant Rosenthal fibers. Many round pTDP-43-positive structures were detected throughout the CNS; however, most of them were located in two regions that also exhibited neuronal depletion: the cerebellar cortex and the inferior olivary nucleus. To a lesser extent, these aggregates were also present in the cerebellar white matter, around the subependymal regions in the brain stem, and in the spinal cord. Intraneuronal pTDP-43 inclusions were only observed in a small number of neurons in the inferior olivary nucleus. Double-label immunofluorescence revealed that many of the aggregates were localized to astrocytes. The observed distribution and the morphology of the pTDP-43-positive structures were unique and have not yet been reported. Therefore, a pTDP-43-related pathology may be implicated in CS as well as in other neurodegenerative diseases such as frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Whether the pathology of these diseases reflects a primary neurodegenerative process or a secondary reaction is not known.
机译：在这里，我们报告了一名日本男子的Cockayne综合征（CS），该病例表现出具有丰富的Rosenthal纤维的磷酸化反式激活反应（TAR）DNA结合蛋白43（pTDP-43）的独特病理。在整个中枢神经系统中发现了许多圆形的pTDP-43阳性结构。然而，它们中的大多数位于两个也表现出神经元耗竭的区域：小脑皮层和下橄榄核。在较小程度上，这些聚集体也存在于小脑白质，脑干的室管膜下区域周围和脊髓中。仅在下橄榄核的少数神经元中观察到神经内pTDP-43夹杂物。双标记免疫荧光显示，许多聚集体定位于星形胶质细胞。 pTDP-43阳性结构的观察到的分布和形态是独特的，尚未有报道。因此，pTDP-43相关的病理可能与CS以及其他神经退行性疾病（如额颞叶变性和肌萎缩性侧索硬化症）有关。这些疾病的病理反应是原发性神经退行性过程还是继发性反应尚不清楚。

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