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Published in:

01-03-2006 | Original Article

Death of neuronal clusters contributes to variance of age at onset in Huntington’s disease

Authors: Branka Čajavec, Hanspeter Herzel, Samuel Bernard

Published in: Neurogenetics | Issue 1/2006

Abstract

Huntington’s disease (HD) is a fatal neurodegenerative disease caused by an expanded polyglutamine (polyQ) repeat in the protein huntingtin. Due to selective neuronal loss in the cortex and striatum, HD patients develop various movement disturbances, psychological changes, and dementia. Symptoms usually appear in individuals between 30 and 50 years of age. The principal cause of variability of age at onset (AO) is the length of the polyQ repeat. Several additional genetic factors contributing to the variance have been identified. At least 35% of the variance, however, remains unexplained. Using a stochastic model, we show that the pattern of cell death of striatal neurons might contribute up to 20% of variance of AO.

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Title: Death of neuronal clusters contributes to variance of age at onset in Huntington’s disease
Authors: Branka Čajavec
Hanspeter Herzel
Samuel Bernard
Publication date: 01-03-2006
Publisher: Springer-Verlag
Published in: Neurogenetics / Issue 1/2006
Print ISSN: 1364-6745
Electronic ISSN: 1364-6753
DOI: https://doi.org/10.1007/s10048-005-0025-x

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Death of neuronal clusters contributes to variance of age at onset in Huntington’s disease

Abstract

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Abstract

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