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Differential loss of striatal projection neurons in Huntington disease.

Reiner, A, Albin, R L, Anderson, K D, D'Amato, C J, Penney, J B, Young, A B·1988·Proceedings of the National Academy of Sciences
DOI 10.1073/pnas.85.15.5733
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Abstract

Huntington disease (HD) is characterized by the loss of striatal projection neurons, which constitute the vast majority of striatal neurons. To determine whether there is differential loss among different populations of striatal projection neurons, the integrity of the axon terminal plexuses arising from the different populations of substance P-containing and enkephalin-containing striatal projection neurons was studied in striatal target areas by immunohistochemistry. Analysis of 17 HD specimens indicated that in early and middle stages of HD, enkephalin-containing neurons projecting to the external segment of the globus pallidus were much more affected than substance P-containing neurons projecting to the internal pallidal segment. Furthermore, substance P-containing neurons projecting to the substantia nigra pars reticulata were more affected than those projecting to the substantia nigra pars compacta. At the most advanced stages of the disease, projections to all striatal target areas were depleted, with the exception of some apparent sparing of the striatal projection to the substantia nigra pars compacta. These findings may explain some of the clinical manifestations and pharmacology of HD. They also may aid in identifying the neural defect underlying HD and provide additional data with which to evaluate current models of HD pathogenesis.

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Authors
Reiner, A., Albin, R. L., Anderson, K. D., D'Amato, C. J., Penney, J. B., & Young, A. B.
Affiliations
Department of Anatomy and Neurobiology, University of Tennessee, Memphis 38163.
Venue
Proceedings of the National Academy of Sciences
Publisher
National Academy of Sciences
Published
1988-08
Volume
85
Issue
15
Pages
5733-5737
ISSN
  • 0027-8424
  • 1091-6490
DOI
10.1073/pnas.85.15.5733
Cited by
839
References
0
Language
en

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