Ribosomal protein SA is a common component of neuronal intranuclear inclusions in polyglutamine diseases and Marinesco bodies.

Autor: Yagita K; Department of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan., Sadashima S; Department of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan., Koyama S; Department of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan., Noguchi H; Department of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan., Hamasaki H; Department of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan., Sasagasako N; Department of Neurology, National Hospital Organization, Omuta National Hospital, Fukuoka, Japan., Honda H; Department of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.; Neuropathology Center, National Hospital Organization, Omuta National Hospital, Omuta, Japan.
Jazyk: angličtina
Zdroj: Neuropathology : official journal of the Japanese Society of Neuropathology [Neuropathology] 2024 Feb; Vol. 44 (1), pp. 31-40. Date of Electronic Publication: 2023 Jun 21.
DOI: 10.1111/neup.12927
Abstrakt: 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.
(© 2023 Japanese Society of Neuropathology.)
Databáze: MEDLINE
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