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Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain

DiFiglia, Marian, Sapp, Ellen, Chase, Kathryn O., Davies, Stephen W., Bates, Gillian P., Vonsattel, J. P., Aronin, Neil·1997·Science
DOI 10.1126/science.277.5334.1990
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Abstract

The cause of neurodegeneration in Huntington's disease (HD) is unknown. Patients with HD have an expanded NH 2 -terminal polyglutamine region in huntingtin. An NH 2 -terminal fragment of mutant huntingtin was localized to neuronal intranuclear inclusions (NIIs) and dystrophic neurites (DNs) in the HD cortex and striatum, which are affected in HD, and polyglutamine length influenced the extent of huntingtin accumulation in these structures. Ubiquitin was also found in NIIs and DNs, which suggests that abnormal huntingtin is targeted for proteolysis but is resistant to removal. The aggregation of mutant huntingtin may be part of the pathogenic mechanism in HD.

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Crossref · 10.1126/science.277.5334.1990

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2 of 2 references verified0 citation errors · 0 not checkable

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Citations

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Metadata

Authors
DiFiglia, M., Sapp, E., Chase, K. O., Davies, S. W., Bates, G. P., Vonsattel, J. P., & Aronin, N.
Affiliations
M. DiFiglia, E. Sapp, J. P. Vonsattel, Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA.
Venue
Science
Publisher
American Association for the Advancement of Science (AAAS)
Published
1997-09-26
Volume
277
Issue
5334
Pages
1990-1993
ISSN
  • 0036-8075
  • 1095-9203
DOI
10.1126/science.277.5334.1990
Cited by
2,439
References
2
Language
en

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