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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Immunohistochemical localization of spatacsin in α-synucleinopathies.
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Immunohistochemical localization of spatacsin in α-synucleinopathies.

机译：Spatacsin在α-突触核蛋白病中的免疫组织化学定位。

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摘要

Spatacsin (SPG11) is a major mutated gene in autosomal recessive spastic paraplegia with thin corpus callosum (ARHSP-TCC) and is responsible for juvenile Parkinsonism. To elucidate the role of spatacsin in the pathogenesis of α-synucleinopathies, an immunohistochemical investigation was performed on the brain of patients with Parkinson's disease (PD), dementia with Lewy bodies (DLB) and multiple system atrophy (MSA) using anti-spatacsin antibody. In PD, Lewy bodies (LBs) in the brain stem were positive for spatacsin. These LBs showed intense staining in their peripheral portions and occasionally in the central cores. Lewy neurites were also spatacsin-positive. In DLB, cortical LBs were immunolabeled by spatacsin. In MSA, glial cytoplasmic inclusions (GCI) and a small fraction of neuronal cytoplasmic inclusions (NCI) were positive for spatacsin. The widespread accumulation of spatacsin observed in pathologic α-synuclein-containing inclusions suggests that spatacsin may be involved in the pathogenesis of α-synucleinopathies.
机译：Spatacsin（SPG11）是常染色体隐性痉挛性截瘫伴稀薄call体（ARHSP-TCC）的主要突变基因，负责青少年帕金森氏病。为了阐明spatacsin在α-突触核蛋白病发病机理中的作用，使用抗spatacsin抗体对帕金森氏病（PD），路易氏体痴呆（DLB）和多系统萎缩（MSA）患者的大脑进行了免疫组织化学研究。在PD中，脑干中的路易体（LB）呈阳性。这些LBs在其外围部分以及偶尔在中央核心中显示出强烈的染色。路易神经突也为斯帕西菌阳性。在DLB中，皮质乳杆菌通过Spatacsin进行免疫标记。在MSA中，神经胶质细胞质内含物（GCI）和一小部分神经元细胞质内含物（NCI）呈阳性。在含有病理性α-突触核蛋白的包裹体中观察到的Spatacsin的广泛积累表明Spatacsin可能参与了α-突触核蛋白致病的发病机理。

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