Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain-Reference-Cited by-同舟云学术

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

Published:1997-09-26 Issue:5334 Volume:277 Page:1990-1993
ISSN:0036-8075
Container-title:Science
language:en
Short-container-title:Science

Author:

DiFiglia Marian¹²³⁴,Sapp Ellen¹²³⁴,Chase Kathryn O.¹²³⁴,Davies Stephen W.¹²³⁴,Bates Gillian P.¹²³⁴,Vonsattel J. P.¹²³⁴,Aronin Neil¹²³⁴

Affiliation:

1. M. DiFiglia, E. Sapp, J. P. Vonsattel, Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA.

2. S. W. Davies, Department of Anatomy and Developmental Biology, University College London, Gower Street, London WC1E, 6BT, UK.

3. G. P. Bates, Division of Medical and Molecular Genetics, UMDS Guy's Hospital, London SE1 7E H, UK.

4. K. O. Chase, N. Aronin, Departments of Medicine and Cell Biology, University of Massachusetts Medical Center, Worcester, MA 01655, USA.

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.

Publisher

American Association for the Advancement of Science (AAAS)

Subject

Multidisciplinary

Reference58 articles.

1. Byers K., Gilles F. H., Fung C., Neurology 23, 561 (1973);

2. Evidence for Degenerative and Regenerative Changes in Neostriatal Spiny Neurons in Huntington's Disease

3. Cudkowicz M., Kowall N. S., Ann. Neurol. 27, 200 (1990);

4. De La Monte S. M., Vonsattel J. P., Richardson E. P., J. Neuropathol. Exp. Neurol. 47, 516 (1988) ;

5. Myers R. H., et al., ibid. 50, 729 (1991);

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