Article
The Neurodevelopmental Hypothesis of Huntington's Disease.
Journal of Huntington's disease - 1 Jan 2020
van der Plas Ellen, Schultz Jordan L, Nopoulos Peg C
Abstract excerpt
The current dogma of HD pathoetiology posits it is a degenerative disease affecting primarily the striatum, caused by a gain of function (toxicity) of the mutant mHTT that kills neurons. However, a growing body of evidence supports an alternative theory in which loss of function may also influence the pathology.This theory is predicated on the notion that HTT is known to be a vital gene for brain development....
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