Axonopathy in Huntington's disease.
(2013) In Experimental Neurology 246(Aug 19). p.62-71- Abstract
- Personality changes, psychiatric disturbances and cognitive abnormalities frequently characterise the prodromal phase in Huntington's disease (HD), a devastating monogenic neurodegenerative disorder manifesting with abnormal motor movements and early death. Selective loss of medium-sized spiny striatal neurons has been related to the onset of motor symptoms but it does not completely explain the psychiatric and cognitive changes that often precede motor abnormalities. Here we review the evidence of synaptic and axonal dysfunction and neurite dystrophy preceding neuronal loss in HD patients and models. We discuss possible mechanisms leading to dysfunction of the axonal and synaptic compartments and identify potential novel targets for... (More)
- Personality changes, psychiatric disturbances and cognitive abnormalities frequently characterise the prodromal phase in Huntington's disease (HD), a devastating monogenic neurodegenerative disorder manifesting with abnormal motor movements and early death. Selective loss of medium-sized spiny striatal neurons has been related to the onset of motor symptoms but it does not completely explain the psychiatric and cognitive changes that often precede motor abnormalities. Here we review the evidence of synaptic and axonal dysfunction and neurite dystrophy preceding neuronal loss in HD patients and models. We discuss possible mechanisms leading to dysfunction of the axonal and synaptic compartments and identify potential novel targets for effective therapeutic intervention. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/3047228
- author
- Li, Jia-Yi LU and Conforti, Laura
- organization
- publishing date
- 2013
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Experimental Neurology
- volume
- 246
- issue
- Aug 19
- pages
- 62 - 71
- publisher
- Elsevier
- external identifiers
-
- wos:000321995700008
- pmid:22921535
- scopus:84879800629
- pmid:22921535
- ISSN
- 0014-4886
- DOI
- 10.1016/j.expneurol.2012.08.010
- language
- English
- LU publication?
- yes
- id
- 436fdc79-6886-4353-96f6-3327589b95a4 (old id 3047228)
- alternative location
- http://www.ncbi.nlm.nih.gov/pubmed/22921535?dopt=Abstract
- date added to LUP
- 2016-04-01 10:16:16
- date last changed
- 2022-04-27 20:22:27
@article{436fdc79-6886-4353-96f6-3327589b95a4,
abstract = {{Personality changes, psychiatric disturbances and cognitive abnormalities frequently characterise the prodromal phase in Huntington's disease (HD), a devastating monogenic neurodegenerative disorder manifesting with abnormal motor movements and early death. Selective loss of medium-sized spiny striatal neurons has been related to the onset of motor symptoms but it does not completely explain the psychiatric and cognitive changes that often precede motor abnormalities. Here we review the evidence of synaptic and axonal dysfunction and neurite dystrophy preceding neuronal loss in HD patients and models. We discuss possible mechanisms leading to dysfunction of the axonal and synaptic compartments and identify potential novel targets for effective therapeutic intervention.}},
author = {{Li, Jia-Yi and Conforti, Laura}},
issn = {{0014-4886}},
language = {{eng}},
number = {{Aug 19}},
pages = {{62--71}},
publisher = {{Elsevier}},
series = {{Experimental Neurology}},
title = {{Axonopathy in Huntington's disease.}},
url = {{https://lup.lub.lu.se/search/files/1699897/3128124.pdf}},
doi = {{10.1016/j.expneurol.2012.08.010}},
volume = {{246}},
year = {{2013}},
}