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Titolo:
Neuronal degeneration in the basal ganglia and loss of pallido-subthalamicsynapses in mice with targeted disruption of the Huntington's disease gene
Autore:
OKusky, JR; Nasir, J; Cicchetti, F; Parent, A; Hayden, MR;
Indirizzi:
Univ British Columbia, Dept Pathol & Lab Med, Vancouver, BC V5Z 1M9, Canada Univ British Columbia Vancouver BC Canada V5Z 1M9 ver, BC V5Z 1M9, Canada Univ British Columbia, Dept Med Genet, Vancouver, BC V6T 1Z4, Canada Univ British Columbia Vancouver BC Canada V6T 1Z4 ver, BC V6T 1Z4, Canada Univdaritish Columbia, Ctr Mol Med & Therapeut, Vancouver, BC V6T 1Z4, Cana Univ British Columbia Vancouver BC Canada V6T 1Z4 ouver, BC V6T 1Z4, Cana Univ,Laval, Ctr Rech Robert Giffard, Neurobiol Lab, Quebec City, PQ G1J 2B3 Univ Laval Quebec City PQ Canada G1J 2B3 iol Lab, Quebec City, PQ G1J 2B3
Titolo Testata:
BRAIN RESEARCH
fascicolo: 2, volume: 818, anno: 1999,
pagine: 468 - 479
SICI:
0006-8993(19990213)818:2<468:NDITBG>2.0.ZU;2-8
Fonte:
ISI
Lingua:
ENG
Soggetto:
INFANT-DEATH-SYNDROME; CELL-DEATH; WIDESPREAD EXPRESSION; EMBRYONIC LETHALITY; REPEAT LENGTH; NUCLEUS; DENSITY; HOMOLOG; TISSUES; PROTEIN;
Keywords:
apoptosis; huntingtin protein; Huntington's disease; subthalamic nucleus;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
40
Recensione:
Indirizzi per estratti:
Indirizzo: O'Kusky, JR Univl,2733sh Columbia, Dept Pathol & Lab Med, Room 364,C Floor,Heather Pavi Univ British Columbia Room 364,C Floor,Heather Pavil,2733 Heather St Vancouver BC Canada V5Z 1M9
Citazione:
J.R. O'Kusky et al., "Neuronal degeneration in the basal ganglia and loss of pallido-subthalamicsynapses in mice with targeted disruption of the Huntington's disease gene", BRAIN RES, 818(2), 1999, pp. 468-479

Abstract

Huntington's disease (HD) is a progressive neurodegenerative disorder associated with CAG repeat expansion within a novel gene (IT15). We have previously created a targeted disruption in exon 5 of Hdh (Hdh(ex5)), the murine homologue of the HD gene. Homozygotes for the Hdh(ex5) mutation exhibit embryolethality before embryonic day 8.5, while heterozygotes survive to adulthood and display increased motor activity and cognitive deficits. Detailed morphometric and stereological analyses of the basal ganglia in adult heterozygous mice were performed by light and electron microscopy. Morphometric analyses demonstrated a significant loss of neurons from both the globus pallidus (29 %) and the subthalamic nucleus (51 %). with a normal complement of neurons in the caudate-putamen and substantia nigra. The ultrastructuralappearance of sporadic degenerating neurons in these regions indicated apoptosis. The highest frequency of apoptotic neurons was observed in the globus pallidus and subthalamic nucleus. Stereological analyses in the subthalamic nucleus revealed a significant decrease in the numerical density of symmetric synapses (43 %), suggesting a relatively selective loss of inhibitory pallido-subthalamic afferents. Immunohistochemistry using antibodies against enkephalin and substance-P was unremarkable in heterozygotes, indicating a normal complement of enkephalin-immunoreactive striatopallidal afferents and substance-P-immunoreactive striatopeduncular and striatonigral afferents in these animals. These findings show that loss of an intact huntingtinprotein is associated with significant morphological alterations in the basal ganglia of adult mice, indicating an important role for this protein during development of the central nervous system. (C) 1999 Elsevier Science B. V. All rights reserved.

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Documento generato il 28/03/20 alle ore 23:32:26