Huntington disease

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dc.contributor.author Kim, EH en
dc.contributor.author Mehrabi, N en
dc.contributor.author Tippett, Lynette en
dc.contributor.author Waldvogel, Henry en
dc.contributor.author Faull, Richard en
dc.contributor.editor Cechetto, DF en
dc.contributor.editor Weishaupt, N en
dc.date.accessioned 2017-09-06T23:52:23Z en
dc.date.accessioned 2020-04-02T23:10:33Z en
dc.date.issued 2017 en
dc.identifier.citation In The cerebral cortex in neurodegenerative and neuropsychiatric disorders: Experimental approaches to clinical issues. Editors: Cechetto DF, Weishaupt N. 195-221. Academic Press, London, UK 2017 en
dc.identifier.isbn 9780128019429 en
dc.identifier.uri http://hdl.handle.net/2292/50200 en
dc.description.abstract This chapter focuses on the involvement of the cerebral cortex in Huntington disease, with special emphasis on the link between cortical pathology and variable clinical manifestations. Huntington disease is an autosomal-dominant neurodegenerative disorder characterized by involuntary movements and cognitive and psychiatric disturbances. The disease is caused by an expansion of polyglutamine repeats in the N-terminal domain of the huntingtin protein from a genetic mutation in the HTT gene on human chromosome 4. The major pathosis occurs in the brain with profound degeneration in the forebrain regions, namely the striatum and the cerebral cortex. Despite the single gene etiology, there is major variability in the neuropathology, as well as major heterogeneity in the symptom profiles. Recent in vivo imaging and pathological studies have shown that cortical degeneration is related to variable symptomatology. Here, we summarize the recent progress on how cortical degeneration contributes to symptom heterogeneity in Huntington disease. en
dc.publisher Academic Press en
dc.relation.ispartof The cerebral cortex in neurodegenerative and neuropsychiatric disorders: Experimental approaches to clinical issues en
dc.relation.replaces http://hdl.handle.net/2292/35535 en
dc.relation.replaces 2292/35535 en
dc.relation.replaces 2292/41327 en
dc.relation.replaces http://hdl.handle.net/2292/41327 en
dc.relation.replaces 2292/41327 en
dc.relation.replaces http://hdl.handle.net/2292/41327 en
dc.relation.replaces 2292/41327 en
dc.relation.replaces http://hdl.handle.net/2292/41327 en
dc.relation.replaces 2292/41327 en
dc.relation.replaces http://hdl.handle.net/2292/41327 en
dc.rights Items in ResearchSpace are protected by copyright, with all rights reserved, unless otherwise indicated. Previously published items are made available in accordance with the copyright policy of the publisher. en
dc.rights.uri https://researchspace.auckland.ac.nz/docs/uoa-docs/rights.htm en
dc.title Huntington disease en
dc.type Book Item en
dc.identifier.doi 10.1016/B978-0-12-801942-9.00008-2 en
pubs.begin-page 195 en
dc.rights.holder Copyright: Academic Press en
pubs.author-url http://www.sciencedirect.com/science/article/pii/B9780128019429000082 en
pubs.end-page 221 en
pubs.merge-from 2292/41327 en
pubs.merge-from http://hdl.handle.net/2292/41327 en
pubs.place-of-publication London, UK en
pubs.publication-status Published en
dc.rights.accessrights http://purl.org/eprint/accessRights/RestrictedAccess en
pubs.elements-id 610857 en
pubs.org-id Medical and Health Sciences en
pubs.org-id Medical Sciences en
pubs.org-id Anatomy and Medical Imaging en
pubs.org-id Pharmacology en
pubs.org-id Science en
pubs.org-id Psychology en
pubs.number 8 en
pubs.record-created-at-source-date 2017-09-07 en


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