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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Valosin-containing protein immunoreactivity in tauopathies, synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease.
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Valosin-containing protein immunoreactivity in tauopathies, synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease.

机译：taopathies，突触核蛋白病，polyglutamine病和核内包涵体病中含有Valosin的蛋白免疫反应性。

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摘要

Valosin-containing protein (VCP) is associated with multiple cellular functions, including ubiquitin-dependent protein degradation. Mutations in VCP are known to cause inclusion body myopathy with Paget's disease and frontotemporal dementia and familial amyotrophic lateral sclerosis (fALS; ALS14), both of which are characterized by trans-activation response DNA protein 43 (TDP-43)-positive neuronal cytoplasmic and nuclear inclusions. Recently, immunoreactivity for fALS-associated proteins (TDP-43, fused in sarcoma (FUS), optineurin and ubiquilin-2) were reported to be present in cytoplasmic and nuclear inclusions in various neurodegenerative diseases. However, the extent and frequency of VCP-immunoreactive structures in these neurodegenerative diseases are uncertain. We immunohistochemically examined the brains of 72 cases with neurodegenerative diseases and five control cases. VCP immunoreactivity was present in Lewy bodies in Parkinson's disease and dementia with Lewy bodies, and neuronal nuclear inclusions in five polyglutamine diseases and intranuclear inclusion body disease, as well as in Marinesco bodies in aged control subjects. However, other neuronal and glial cytoplasmic inclusions in tauopathies and TDP-43 proteinopathies were unstained. These findings suggest that VCP may have common mechanisms in the formation or degradation of cytoplasmic and nuclear inclusions of neurons, but not of glial cells, in several neurodegenerative conditions.
机译：含Valosin的蛋白质（VCP）与多种细胞功能有关，包括泛素依赖性蛋白质降解。已知VCP的突变会引起佩吉特氏病和额颞叶痴呆和家族性肌萎缩性侧索硬化（fALS; ALS14）的包涵体肌病，这两种疾病均以反式激活反应DNA蛋白43（TDP-43）阳性的神经元胞浆和核包裹体。最近，据报道，与fALS相关的蛋白质（TDP-43，肉瘤（FUS），optineurin和ubiquilin-2融合）的免疫反应性存在于多种神经退行性疾病的细胞质和核内。但是，这些神经退行性疾病中VCP免疫反应结构的程度和频率尚不确定。我们通过免疫组织化学检查了72例神经退行性疾病和5例对照病例的大脑。 VCP免疫反应性存在于帕金森氏病和痴呆中的路易体中，并伴有路易体，五种多谷氨酰胺疾病和核内包涵体病中的神经元核内含物，以及老年对照受试者的马里斯科病体中也存在。然而，taopathy和TDP-43蛋白病中的其他神经元和神经胶质细胞质包裹体未染色。这些发现表明，在某些神经退行性疾病中，VCP可能在神经元而非胶质细胞的细胞质和核内含物的形成或降解中具有共同的机制。

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