Hunting in the calm before the storm

Features of a mouse model of early-stage Huntington disease indicate that the model is reasonably accurate. An amino-terminal fragment of huntingtin, detected by western blot, is consistent with an initial pathogenic processing event and nuclear and extranuclear polyglutamine aggregates form selectively within striatal neurons. Further analysis of the model implies an extranuclear mechanism of neuronal dysfunction involving decreased uptake of glutamate by synaptic vesicles.

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Bates, G., Eberwine, J. Hunting in the calm before the storm. Nat Genet 25, 365–366 (2000). https://doi.org/10.1038/78020

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