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散发性运动神经元病中的路易小体样透明包涵体发生了泛素化。

Lewy body-like hyaline inclusions in sporadic motor neuron disease are ubiquitinated.

作者信息

Kato T, Katagiri T, Hirano A, Kawanami T, Sasaki H

机构信息

Third Department of Internal Medicine, Yamagata University School of Medicine, Japan.

出版信息

Acta Neuropathol. 1989;77(4):391-6. doi: 10.1007/BF00687373.

DOI:10.1007/BF00687373
PMID:2469287
Abstract

Round eosinophilic hyaline inclusion bodies with halos in the somata of anterior horn cells from a case of sporadic lower motor neuron disease (MND) were intensely immunostained with the monoclonal anti-ubiquitin antibody (DF2). A few similar, DF2-positive inclusions were also observed in the nerve cell processes of anterior horn cell or in the neuropil. Most inclusions showed intense homogeneous staining of the entire inclusion, whereas a few had intense staining of their periphery with no or pale staining of the central areas. Other DF2-positive structures in the somata of anterior horn cells included cytoplasmic granular structures, eosinophilic thread-like or reticular structures, and small eosinophilic profiles different from Bunina bodies. The DF2-staining intensity of Bunina bodies and spheroids did not exceed the background level. These results suggest that ubiquitination is associated with a pathological process of anterior horn cell degeneration in this MND case.

摘要

在一例散发性下运动神经元疾病(运动神经元病,MND)中,前角细胞胞体中的圆形嗜酸性透明包涵体周围有光晕,用单克隆抗泛素抗体(DF2)进行免疫染色时呈强阳性。在前角细胞的神经突或神经纤维网中也观察到一些类似的DF2阳性包涵体。大多数包涵体整个包涵体呈现强而均匀的染色,而少数包涵体周边染色强,中央区域无染色或染色浅。前角细胞胞体中其他DF2阳性结构包括细胞质颗粒结构、嗜酸性丝状或网状结构,以及不同于布尼纳小体的小嗜酸性结构。布尼纳小体和球体的DF2染色强度未超过背景水平。这些结果表明,在该MND病例中,泛素化与前角细胞变性的病理过程相关。

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