Differential loss of striatal projection neurons in Huntington disease

Journal Article · Mon Aug 01 04:00:00 EDT 1988 · Proceedings of the National Academy of Sciences of the United States of America; (USA)

DOI:https://doi.org/10.1073/pnas.85.15.5733· OSTI ID:5404940

Reiner, A; Albin, R L; Anderson, K D; D'Amato, C J; Penney, J B; Young, A B ^[1]

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1. Univ. of Tennessee, Memphis (USA)

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 finding 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.

• OSTI ID: 5404940
• Journal Information: Proceedings of the National Academy of Sciences of the United States of America; (USA), Journal Name: Proceedings of the National Academy of Sciences of the United States of America; (USA) Vol. 85:15; ISSN 0027-8424; ISSN PNASA
• Country of Publication: United States
• Language: English

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550400* -- Genetics
59 BASIC BIOLOGICAL SCIENCES
ANIMAL CELLS
BIOCHEMISTRY
BODY
BRAIN
CENTRAL NERVOUS SYSTEM
CHEMISTRY
CYTOCHEMISTRY
DISEASES
HEREDITARY DISEASES
IMMUNOLOGY
NERVE CELLS
NERVOUS SYSTEM
NERVOUS SYSTEM DISEASES
ORGANS
PATHOGENESIS
PATIENTS
PHARMACOLOGY
SOMATIC CELLS