Visualization of Prion-Like Transfer in Huntington's Disease Models

Biochim Biophys Acta Mol Basis Dis. 2017 Mar;1863(3):793-800. doi: 10.1016/j.bbadis.2016.12.015. Epub 2016 Dec 29.

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.

Keywords: Aggregation; Huntington; Microscopy; Neurodegeneration; Prion.

Publication types

  • Review

MeSH terms

  • Animals
  • Humans
  • Huntingtin Protein / analysis
  • Huntingtin Protein / genetics
  • Huntingtin Protein / metabolism*
  • Huntington Disease / genetics
  • Huntington Disease / metabolism*
  • Huntington Disease / pathology
  • Mutation
  • Neuroglia / metabolism*
  • Neuroglia / pathology
  • Neurons / metabolism*
  • Neurons / pathology
  • Prions / analysis
  • Prions / genetics
  • Prions / metabolism*
  • Protein Aggregates
  • Protein Aggregation, Pathological / genetics
  • Protein Aggregation, Pathological / metabolism*
  • Protein Aggregation, Pathological / pathology
  • Protein Transport

Substances

  • Huntingtin Protein
  • Prions
  • Protein Aggregates