title: Visualization of prion-like transfer in Huntington's disease models.
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.
authors.affiliation: Department of Cell Biology & Histology, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: Anne H P
authors.initials: AH
authors.lastname: Jansen
authors.affiliation: Department of Cell Biology & Histology, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: Kevin L
authors.initials: KL
authors.lastname: Batenburg
authors.affiliation: Department of Biomedicine, University of Basel and University Hospital Basel, Basel, Switzerland.
authors.forename: Eline
authors.initials: E
authors.lastname: Pecho-Vrieseling
authors.affiliation: Department of Cell Biology & Histology, Academic Medical Center, Amsterdam, The Netherlands. Electronic address: e.a.reits@amc.uva.nl.
authors.forename: Eric A
authors.initials: EA
authors.lastname: Reits
mesh_terms: D000818:Animals; D006801:Humans; D000071058:Huntingtin Protein / Q000032:analysis / Q000235:genetics / Q000378:metabolism*; D006816:Huntington Disease / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D009154:Mutation; D009457:Neuroglia / Q000378:metabolism* / Q000473:pathology; D009474:Neurons / Q000378:metabolism* / Q000473:pathology; D011328:Prions / Q000032:analysis / Q000235:genetics / Q000378:metabolism*; D066329:Protein Aggregates; D066263:Protein Aggregation, Pathological / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D021381:Protein Transport
chemical_list: D000071058:Huntingtin Protein; D011328:Prions; D066329:Protein Aggregates
keywords: Aggregation; Huntington; Microscopy; Neurodegeneration; Prion