核糖体蛋白 SA 是多聚谷氨酰胺疾病和马林斯科体中神经元核内包涵体的常见成分。

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Neuropathology Pub Date : 2024-02-01 Epub Date: 2023-06-21 DOI:10.1111/neup.12927
Kaoru Yagita, Shoko Sadashima, Sachiko Koyama, Hideko Noguchi, Hideomi Hamasaki, Naokazu Sasagasako, Hiroyuki Honda
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引用次数: 0

摘要

神经元核内包涵体(NII)是多谷氨酰胺(polyQ)疾病(如亨廷顿病(HD)、脊髓小脑共济失调 1 型(SCA1)和 SCA3)中常见的关键结构。黑质中多巴胺能神经元的马林斯科体(MBs)也是核内结构,在正常老年人中经常出现。核糖体功能障碍与这两种不同的过程密切相关;因此,我们旨在确定核糖体蛋白 SA(RPSA)在这两种状态下的病理特征。为此,我们对四名 HD 患者、两名 SCA3 患者和五名正常老年病例(NCs)的尸检结果进行了评估。免疫组化研究表明,NII 和 MB 均含有 RPSA。在多Q疾病中,RPSA与多Q聚集体共定位,三维重建图像显示了其马赛克样分布。通过评估RPSA和p62在NIIs中的组织结构,发现RPSA比p62更多地向中心定位,这种独特的组织结构在MBs中更为明显。颞叶皮层的免疫印迹显示,HD 患者的核部分比 NCs 中含有更多的 RPSA。总之,我们的研究发现,RPSA 是 NIIs 和 MBs 的共同成分,这表明多 Q NIIs 和 MBs 的形成有类似的机制。
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Ribosomal protein SA is a common component of neuronal intranuclear inclusions in polyglutamine diseases and Marinesco bodies.

Neuronal intranuclear inclusions (NIIs) are common key structures in polyglutamine (polyQ) diseases such as Huntington disease (HD), spinocerebellar ataxia type 1 (SCA1), and SCA3. Marinesco bodies (MBs) of dopaminergic neurons in the substantia nigra are also intranuclear structures and are frequently seen in normal elderly people. Ribosomal dysfunction is closely related to two differential processes; therefore, we aimed to identify the pathological characteristics of ribosomal protein SA (RPSA), a ribosomal protein, in both states. To this end, we evaluated the autopsy findings in four patients with HD, two SCA3, and five normal elderly cases (NCs). Immunohistochemical studies demonstrated that both NIIs and MBs contain RPSA. In polyQ diseases, RPSA was co-localized with polyQ aggregations, and 3D-reconstructed images revealed their mosaic-like distribution. Assessments of the organization of RPSA and p62 in NIIs showed that RPSA was more localized toward the center than p62 and that this unique organization was more evident in the MBs. Immunoblotting of the temporal cortices revealed that the nuclear fraction of HD patients contained more RPSA than that of NCs. In conclusion, our study revealed that RPSA is a common component of both NIIs and MBs, indicating that a similar mechanism contributes to the formation of polyQ NIIs and MBs.

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来源期刊
Neuropathology
Neuropathology 医学-病理学
CiteScore
4.10
自引率
4.30%
发文量
105
审稿时长
6-12 weeks
期刊介绍: Neuropathology is an international journal sponsored by the Japanese Society of Neuropathology and publishes peer-reviewed original papers dealing with all aspects of human and experimental neuropathology and related fields of research. The Journal aims to promote the international exchange of results and encourages authors from all countries to submit papers in the following categories: Original Articles, Case Reports, Short Communications, Occasional Reviews, Editorials and Letters to the Editor. All articles are peer-reviewed by at least two researchers expert in the field of the submitted paper.
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