期刊
PATHOLOGY INTERNATIONAL
卷 62, 期 6, 页码 407-411出版社
WILEY
DOI: 10.1111/j.1440-1827.2012.02812.x
关键词
intranuclear inclusion body disease; NEDD8; NUB1; SUMO; ubiquitin
类别
资金
- Ministry of Education, Culture, Sports, Science, and Technology, Japan
- Research Committee for Ataxic Disease
- Research Committee of CNS Degenerative Diseases
- Ministry of Health, Labour and Welfare of Japan
- NCNP [21B-4]
- National Institutes of Health [R01AG024497]
- Grants-in-Aid for Scientific Research [23500424] Funding Source: KAKEN
Recent studies have shown that eosinophilic intranuclear inclusions (INI) in the brain of patients with intranuclear inclusion body disease (INIBD) are immunopositive for ubiquitin and ubiquitin-related proteins (URP). However, the extent and frequency of URP-immunoreactive inclusions in INIBD are uncertain. We immunohistochemically examined the brain, spinal cord and dorsal root ganglia from five patients with INIBD, using a virtual slide system with sequential staining of the same sections with hematoxylin and eosin and by immunolabeling with antibodies against ubiquitin and URP (NEDD8, NUB1, SUMO-1 and SUMO-2). Intranuclear inclusions were widely distributed in neurons and glial cells in all the cases. Sequential staining revealed that 100% of INI in neurons and glial cells were positive for ubiquitin. Moreover, the majority or a significant proportion of INI were positive for NEDD8, NUB1, SUMO-1 and SUMO-2. However, the proportions of NEDD8-, NUB1- and SUMO-1-positive inclusions were significantly higher in neurons than in glial cells (P < 0.05). These findings suggest that proteins related to ubiquitination and proteasomal degradation are involved in the formation of INI in INIBD.
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