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Animals; Humans; Huntingtin Protein, metabolism; Huntington Disease, pathology; Mutation; Neuroglia, pathology; Neurons, pathology; Prions, metabolism; Protein Aggregates; Protein Aggregation, Pathological, pathology; Protein Transport
Abstract
Most neurodegenerative diseases such as Alzheimer's, Parkinson's and Huntington's disease are hallmarked by aggregate formation of disease-related proteins. In various of these diseases transfer of aggregation-prone proteins between neurons and between neurons and glial cells has been shown, thereby initiating aggregation in neighboring cells and so propagating the disease phenotype. Whereas this prion-like transfer is well studied in Alzheimer's and Parkinson's disease, only a few studies have addressed this potential mechanism in Huntington's disease. Here, we present an overview of in vitro and in vivo methodologies to study release, intercellular transfer and uptake of aggregation-prone protein fragments in Huntington's disease models.
