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Axonopathy in Huntington's disease.

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Author

Summary, in English

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.

Publishing year

2013

Language

English

Pages

62-71

Publication/Series

Experimental Neurology

Volume

246

Issue

Aug 19

Document type

Journal article review

Publisher

Elsevier

Topic

  • Neurology

Status

Published

Research group

  • Neural Plasticity and Repair

ISBN/ISSN/Other

  • ISSN: 0014-4886