Neuronal intranuclear hyaline inclusion disease.

Neuronal intranuclear hyaline inclusion disease. Research Abstract Details 

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Neuronal intranuclear hyaline inclusion disease. Abstract Text:

Neuronal intranuclear hyaline inclusion disease (NIHID) is a rare neurodegenerative disorder characterized pathologically by the presence of eosinophilic intranuclear inclusions in neuronal and glial cells. It has been considered to be a heterogeneous disease entity because the clinical pictures of previously described cases were highly variable. In the present review, reported NIHID cases have been categorized into three clinical subgroups according to onset and disease duration, and the clinical phenotype of each subgroup is discussed. Neuronal intranuclear inclusions (NII) in NIHID are ubiquitinated and their prevalence is inversely correlated with neuronal loss, suggesting that NII formation is a protective mechanism involving the ubiquitin-proteasome-dependent proteolytic pathway. In several polyglutamine diseases, disease-related proteins containing abnormally expanded polyglutamine tracts aggregate in neuronal nuclei, resulting in NII formation. The similarity between NII in NIHID and polyglutamine diseases suggests that they are formed during a common proteolysis-related process that takes place in the nucleus. Although the pathogenetic mechanism underlying NIHID remains unknown, the data reviewed here suggest that it might be related to accumulation of as yet unidentified abnormal proteins or dysfunction of the intranuclear ubiquitin-proteasome pathway.

Neuronal intranuclear hyaline inclusion disease. Publishing Authors By Initials

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Neuronal intranuclear hyaline inclusion disease. Journal Published:

PUBLICATION TYPE: Review

Journal: Neuropathology : official journal of the Japanese

VOLUME: 23

Page Numbers: 351-9

Journal Abbreviation: Neuropathology

ISSN: 0919-6544

DAY: 10

MONTH: Dec

YEAR: 2003

Neuronal intranuclear hyaline inclusion disease. Information

Number of References: 57

LANGUAGE: eng

NlmUniqueID: 9606526

Neuronal intranuclear hyaline inclusion disease. Keywords Mesh Terms:

KEYWORDS: Peptides

MESH TERMS: metabolism

Chemical & Substance for Abstract: Neuronal intranuclear hyaline inclusion disease. Information

Substance Name: polyglutamine

Registry Number: 26700-71-0

Grant and Affiliation Information for Neuronal intranuclear hyaline inclusion disease.

AFFILIATION: Division of Neuropathology, Jikei University School of Medicine, Tokyo, Japan. jnk@jikei.ac.jp

Country: Australia

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MEDLINETA: Neuropathology

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