Article
Atg4b-dependent autophagic flux alleviates Huntington's disease progression.
PloS one - 1 Jan 2013
Proenca Catia C, Stoehr Natacha, Bernhard Mario, Seger Shanon, Genoud Christel, Roscic Ana, Paganetti Paolo, Liu Shanming, Murphy Leon O, Kuhn Rainer, Bouwmeester Tewis, Galimberti Ivan
Abstract excerpt
The accumulation of aggregated mutant huntingtin (mHtt) inclusion bodies is involved in Huntigton's disease (HD) progression. Medium sized-spiny neurons (MSNs) in the corpus striatum are highly vulnerable to mHtt aggregate accumulation and degeneration, but the mechanisms and pathways involved remain elusive. Here we have developed a new model to study MSNs degeneration in the context of HD. We produced...
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