Synaptic pathology in Huntington's disease: Beyond the corticostriatal pathway

Huntington's disease (HD) is a heritable, fatal neurodegenerative disorder caused by a mutation in the Huntingtin gene. It is characterized by chorea, as well as cognitive and psychiatric symptoms. Histopathologically, there is a massive loss of striatal projection neurons and less but signific...

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Autores principales: Joshua Barry, Minh T.N. Bui, Michael S. Levine, Carlos Cepeda
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Publicado: Elsevier 2022
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spelling oai:doaj.org-article:15c95e1409ae43cdaff29b2eb915034a2021-12-02T04:59:16ZSynaptic pathology in Huntington's disease: Beyond the corticostriatal pathway1095-953X10.1016/j.nbd.2021.105574https://doaj.org/article/15c95e1409ae43cdaff29b2eb915034a2022-01-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S0969996121003235https://doaj.org/toc/1095-953XHuntington's disease (HD) is a heritable, fatal neurodegenerative disorder caused by a mutation in the Huntingtin gene. It is characterized by chorea, as well as cognitive and psychiatric symptoms. Histopathologically, there is a massive loss of striatal projection neurons and less but significant loss in other areas throughout the cortico-basal ganglia-thalamocortical (CBGTC) loop. The mutant huntingtin protein has been implicated in numerous functions, including an important role in synaptic transmission. Most studies on anatomical and physiological alterations in HD have focused on striatum and cerebral cortex. However, based on recent CBGTC projectome evidence, the need to study other pathways has become increasingly clear. In this review, we examine the current status of our knowledge of morphological and electrophysiological alterations of those pathways in animal models of HD. Based on recent studies, there is accumulating evidence that synaptic disconnection, particularly along excitatory pathways, is pervasive and almost universal in HD, thus supporting a critical role of the huntingtin protein in synaptic transmission.Joshua BarryMinh T.N. BuiMichael S. LevineCarlos CepedaElsevierarticleHuntington's diseaseGenetic modelsBasal gangliaSynaptic activityDisconnectionNeurosciences. Biological psychiatry. NeuropsychiatryRC321-571ENNeurobiology of Disease, Vol 162, Iss , Pp 105574- (2022)
institution DOAJ
collection DOAJ
language EN
topic Huntington's disease
Genetic models
Basal ganglia
Synaptic activity
Disconnection
Neurosciences. Biological psychiatry. Neuropsychiatry
RC321-571
spellingShingle Huntington's disease
Genetic models
Basal ganglia
Synaptic activity
Disconnection
Neurosciences. Biological psychiatry. Neuropsychiatry
RC321-571
Joshua Barry
Minh T.N. Bui
Michael S. Levine
Carlos Cepeda
Synaptic pathology in Huntington's disease: Beyond the corticostriatal pathway
description Huntington's disease (HD) is a heritable, fatal neurodegenerative disorder caused by a mutation in the Huntingtin gene. It is characterized by chorea, as well as cognitive and psychiatric symptoms. Histopathologically, there is a massive loss of striatal projection neurons and less but significant loss in other areas throughout the cortico-basal ganglia-thalamocortical (CBGTC) loop. The mutant huntingtin protein has been implicated in numerous functions, including an important role in synaptic transmission. Most studies on anatomical and physiological alterations in HD have focused on striatum and cerebral cortex. However, based on recent CBGTC projectome evidence, the need to study other pathways has become increasingly clear. In this review, we examine the current status of our knowledge of morphological and electrophysiological alterations of those pathways in animal models of HD. Based on recent studies, there is accumulating evidence that synaptic disconnection, particularly along excitatory pathways, is pervasive and almost universal in HD, thus supporting a critical role of the huntingtin protein in synaptic transmission.
format article
author Joshua Barry
Minh T.N. Bui
Michael S. Levine
Carlos Cepeda
author_facet Joshua Barry
Minh T.N. Bui
Michael S. Levine
Carlos Cepeda
author_sort Joshua Barry
title Synaptic pathology in Huntington's disease: Beyond the corticostriatal pathway
title_short Synaptic pathology in Huntington's disease: Beyond the corticostriatal pathway
title_full Synaptic pathology in Huntington's disease: Beyond the corticostriatal pathway
title_fullStr Synaptic pathology in Huntington's disease: Beyond the corticostriatal pathway
title_full_unstemmed Synaptic pathology in Huntington's disease: Beyond the corticostriatal pathway
title_sort synaptic pathology in huntington's disease: beyond the corticostriatal pathway
publisher Elsevier
publishDate 2022
url https://doaj.org/article/15c95e1409ae43cdaff29b2eb915034a
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AT minhtnbui synapticpathologyinhuntingtonsdiseasebeyondthecorticostriatalpathway
AT michaelslevine synapticpathologyinhuntingtonsdiseasebeyondthecorticostriatalpathway
AT carloscepeda synapticpathologyinhuntingtonsdiseasebeyondthecorticostriatalpathway
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