The Lewy body in Parkinson's disease and related neurodegenerative disorders

Mol Neurobiol. 2013 Apr;47(2):495-508. doi: 10.1007/s12035-012-8280-y. Epub 2012 May 24.

Abstract

The histopathological hallmark of Parkinson's disease (PD) is the presence of fibrillar aggregates referred to as Lewy bodies (LBs), in which α-synuclein is a major constituent. Pale bodies, the precursors of LBs, may serve the material for that LBs continue to expand. LBs consist of a heterogeneous mixture of more than 90 molecules, including PD-linked gene products (α-synuclein, DJ-1, LRRK2, parkin, and PINK-1), mitochondria-related proteins, and molecules implicated in the ubiquitin-proteasome system, autophagy, and aggresome formation. LB formation has been considered to be a marker for neuronal degeneration because neuronal loss is found in the predilection sites for LBs. However, recent studies have indicated that nonfibrillar α-synuclein is cytotoxic and that fibrillar aggregates of α-synuclein (LBs and pale bodies) may represent a cytoprotective mechanism in PD.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Humans
  • Lewy Bodies / pathology*
  • Lewy Bodies / physiology
  • Lewy Body Disease / metabolism
  • Lewy Body Disease / pathology
  • Neurodegenerative Diseases / metabolism
  • Neurodegenerative Diseases / pathology
  • Neurodegenerative Diseases / prevention & control
  • Parkinson Disease / metabolism*
  • Parkinson Disease / pathology*
  • Parkinson Disease / prevention & control
  • alpha-Synuclein / adverse effects
  • alpha-Synuclein / toxicity

Substances

  • alpha-Synuclein