Neuronal inclusions in degenerative disorders: Do they represent static features or a key to understand the dynamics of the disease?

Francesco Fornai, Paola Soldani, Gloria Lazzeri, Adolfo Bandettini Di Poggio, Francesca Biagioni, Federica Fulceri, Serena Batini, Stefano Ruggieri, Antonio Paparelli

Research output: Contribution to journalArticlepeer-review

Abstract

This brief paper analyzes a few degenerative diseases expressing as movement disorders and featuring at sub-cellular level the presence of neuronal inclusions in selective brain regions. We will first draw a short draft of representative neurological diseases featuring inclusion bodies by describing the type of inclusions occurring in various disorders and analyzing both common features and distinctive aspects. As a further step, we move from the bed to the bench side discussing recent developments obtained from experimental models of these disorders which shed new light into the cause and progression of neuronal inclusions, thus helping to understand the pathophysiology of neuronal degeneration underlying movement disorders. In line with this, we will focus on recent studies which led to reproduce neuronal inclusions in vivo and in vitro by manipulating selective cellular structures/enzymatic pathways. In this way, we will try to encompass the dynamics of inclusion formation based on their fine ultrastructure and the analysis of the molecular components as well as their subcellular compartmentalization trying to relate the dynamics of inclusion formation and the pathophysiology of the disease process. An emphasis will be made on the ubiquitin proteasome system and Parkinson's disease where the analysis of neuronal inclusions enlightened potential therapeutic strategies to occlude the progression of this neuronal degeneration featured by movement disorders.

Original languageEnglish
Pages (from-to)275-290
Number of pages16
JournalBrain Research Bulletin
Volume65
Issue number4
DOIs
Publication statusPublished - Apr 30 2005

Keywords

  • Huntington's disease
  • Lewy bodies
  • Movement disorders
  • Neurodegeneration
  • Neuronal inclusions
  • Parkinson's disease
  • Ubiquitin-proteasome system

ASJC Scopus subject areas

  • Neuroscience(all)

Fingerprint Dive into the research topics of 'Neuronal inclusions in degenerative disorders: Do they represent static features or a key to understand the dynamics of the disease?'. Together they form a unique fingerprint.

Cite this