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Cited by 433

The DOI matches its Crossref record; 3 of the 3 references it cites were found in a registry or an index.

Intranuclear Neuronal Inclusions in Huntington's Disease and Dentatorubral and Pallidoluysian Atrophy: Correlation between the Density of Inclusions andIT15CAG Triplet Repeat Length

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Becher, Mark W., Kotzuk, Joyce A., Sharp, Alan H., Davies, Stephen W., Bates, Gillian P., Price, Donald L., Ross, Christopher A.·1998·Neurobiology of Disease

Abstract

Huntington's disease (HD) is caused by CAG triplet repeat expansion in IT15 which leads to polyglutamine stretches in the HD protein product, huntingtin. The pathological hallmark of HD is the degeneration of subsets of neurons, primarily those in the striatum and neocortex. Specific morphological markers of affected cells have not been identified in patients with HD, although a unique itranuclear inclusion was recently reported in neurons of transgenic animals expressing a construct encoding the N-terminal part (including the glutamine repeat) of huntingtin (Davies et al., 1997). In order to understand the importance of this finding, we sought for comparable nuclear abnormalities in autopsy material from patients with HD. In all 20 HD cases examined, anti-ubiquitin and N-terminal huntingtin antibodies identified itranuclear inclusions in neurons and the frequency of these lesions correlated with the length of the CAG repeat in IT15. In addition, examination of material from the related HD-like triplet repeat disorder, dentatorubral and pallidoluysian atrophy, also revealed intranuclear neuronal inclusions. These findings suggest that intranuclear inclusions containing protein aggregates may be common feature of the pathogenesis of glutamine repeat neurodegenerative disorders.

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Metadata

Authors
Becher, M. W., Kotzuk, J. A., Sharp, A. H., Davies, S. W., Bates, G. P., Price, D. L., & Ross, C. A.
Venue
Neurobiology of Disease
Publisher
Elsevier BV
Published
1998-01
Volume
4
Issue
6
Pages
387-397
ISSN
0969-9961 (ISSN-L)
OpenAlex
W2081595285
Cited by
433
References
3
FWCI
16.70
Type
journal article
Language
en
Open access
hybrid

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CoChat. (2026). Citation check: Intranuclear Neuronal Inclusions in Huntington's Disease and Dentatorubral and Pallidoluysian Atrophy: Correlation between the Density of Inclusions andIT15CAG Triplet Repeat Length. https://app.cochat.ai/papers/10.1006/nbdi.1998.0168
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Becher, M. W., Kotzuk, J. A., Sharp, A. H., Davies, S. W., Bates, G. P., Price, D. L., & Ross, C. A. (1998). Intranuclear Neuronal Inclusions in Huntington's Disease and Dentatorubral and Pallidoluysian Atrophy: Correlation between the Density of Inclusions andIT15CAG Triplet Repeat Length. Neurobiology of Disease. https://doi.org/10.1006/nbdi.1998.0168

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

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