Visualization of prion-like transfer in Huntington's disease models

Anne H.P. Jansen, Kevin L. Batenburg, Eline Pecho-Vrieseling, Eric A. Reits

Research output: Contribution to journalReview articleAcademicpeer-review

18 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)793-800
Number of pages8
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Volume1863
Issue number3
Early online date2016
DOIs
Publication statusPublished - 1 Mar 2017

Keywords

  • Aggregation
  • Huntington
  • Microscopy
  • Neurodegeneration
  • Prion

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