Transmission of Soluble and Insoluble α-Synuclein to Mice

J Neuropathol Exp Neurol. 2015 Dec;74(12):1158-69. doi: 10.1097/NEN.0000000000000262.

Abstract

The neurodegenerative synucleinopathies, which include Parkinson disease, multiple-system atrophy, and Lewy body disease, are characterized by the presence of abundant neuronal inclusions called Lewy bodies and Lewy neurites. These disorders remain incurable, and a greater understanding of the pathologic processes is needed for effective treatment strategies to be developed. Recent data suggest that pathogenic misfolding of the presynaptic protein, α-synuclein (α-syn), and subsequent aggregation and accumulation are fundamental to the disease process. It is hypothesized that the misfolded isoform is able to induce misfolding of normal endogenous α-syn, much like what occurs in the prion diseases. Recent work highlighting the seeding effect of pathogenic α-syn has largely focused on the detergent-insoluble species of the protein. In this study, we performed intracerebral inoculations of the sarkosyl-insoluble or sarkosyl-soluble fractions of human Lewy body disease brain homogenate and show that both fractions induce CNS pathology in mice at 4 months after injection. Disease-associated deposits accumulated both near and distal to the site of the injection, suggesting a cell-to-cell spread via recruitment of α-syn. These results provide further insight into the prion-like mechanisms of α-syn and suggest that disease-associated α-syn is not homogeneous within a single patient but might exist in both soluble and insoluble isoforms.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adaptation, Ocular / genetics
  • Age Factors
  • Aged
  • Animals
  • Brain / drug effects
  • Brain / metabolism*
  • Brain / pathology
  • Brain / ultrastructure
  • Calcium-Binding Proteins / metabolism
  • Detergents / pharmacology
  • Disease Models, Animal
  • Exploratory Behavior / physiology
  • Female
  • Glial Fibrillary Acidic Protein / metabolism
  • Humans
  • Lewy Body Disease / chemically induced*
  • Lewy Body Disease / pathology
  • Lewy Body Disease / physiopathology
  • Lewy Body Disease / therapy
  • Male
  • Maze Learning / physiology
  • Mice
  • Mice, Transgenic
  • Microfilament Proteins / metabolism
  • Microscopy, Electron
  • Muscle Strength / genetics
  • Platelet-Derived Growth Factor / genetics
  • Platelet-Derived Growth Factor / metabolism
  • Sarcosine / analogs & derivatives
  • Sarcosine / pharmacology
  • alpha-Synuclein / administration & dosage
  • alpha-Synuclein / drug effects
  • alpha-Synuclein / genetics
  • alpha-Synuclein / metabolism*

Substances

  • Aif1 protein, mouse
  • Calcium-Binding Proteins
  • Detergents
  • Glial Fibrillary Acidic Protein
  • Microfilament Proteins
  • Platelet-Derived Growth Factor
  • alpha-Synuclein
  • sarkosyl
  • Sarcosine