PerspectiveNeuroscience

An early start to Huntington's disease

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Science  14 Aug 2020:
Vol. 369, Issue 6505, pp. 771-772
DOI: 10.1126/science.abd6215

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Summary

Huntington's disease (HD) is a rare, inherited brain disorder that causes progressive degeneration of neurons, impaired movement and cognition, and death ∼15 years after onset. Most carriers of the pathogenic mutation in the huntingtin (HTT) gene develop symptoms in midlife, but abnormalities in the brain can occur a decade earlier. On page 787 of this issue, Barnat et al. (1) describe anomalies in neuronal precursors destined for the cortex of human fetal brain and embryonic mouse brain harboring HD-associated mutations in the HTT gene. These findings prompt questions about the impact of these events on early development, the emergence of disease, and the timing of therapeutic interventions.

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