Mutant huntingtin (mHTT) might spread from neuron to neuron in a prion-like fashion. Pecho-Vrieseling et al. discovered that neurons derived from human stem cells could acquire mHTT aggregates and HD-like pathology when cultured with mouse organotypic slices that were genetically engineered to recapitulate HD pathophysiology, including mHTT expression. The results suggest that prion-like propagation of mHTT through synaptic vesicle trafficking might contribute to HD pathophysiology.
References
Pecho-Vrieseling, E. et al. Transneuronal propagation of mutant huntingtin contributes to non-cell autonomous pathology in neurons. Nat. Neurosci. 10.1038/nn.3761
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Transneuronal propagation of mutant huntingtin protein. Nat Rev Neurol 10, 427 (2014). https://doi.org/10.1038/nrneurol.2014.132
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DOI: https://doi.org/10.1038/nrneurol.2014.132
