Dysregulation of axonal transport and motorneuron diseases

Daniela Sau, Paola Rusmini, Valeria Crippa, Elisa Onesto, Elena Bolzoni, Antonia Ratti, Angelo Poletti

Research output: Contribution to journalArticlepeer-review

Abstract

MNDs (motorneuron diseases) are neurodegenerative disorders in whichmotorneurons located in themotor cortex, in the brainstem and in the spinal cord are affected. These diseases in their inherited or sporadic forms are mainly characterized by motor dysfunctions, occasionally associated with cognitive and behavioural alterations. Although these diseases show high variability in onset, progression and clinical symptoms, they share common pathological features, and motorneuronal loss invariably leads to muscle weakness and atrophy. One of the most relevant aspect of these disorders is the occurrence of defects in axonal transport, which have been postulated to be either a direct cause, or a consequence, of motorneuron degeneration. In fact, due to their peculiar morphology and high energetic metabolism, motorneurons deeply rely on efficient axonal transport processes. Dysfunction of axonal transport is known to adversely affect motorneuronal metabolism, inducing progressive degeneration and cell death. In this regard, the understanding of the fine mechanisms at the basis of the axonal transport process and of their possible alterations may help shed light on MND pathological processes. In the present review, we will summarize what is currently known about the alterations of axonal transport found to be either causative or a consequence of MNDs.

Original languageEnglish
Pages (from-to)87-107
Number of pages21
JournalBiology of the cell / under the auspices of the European Cell Biology Organization
Volume103
Issue number2
DOIs
Publication statusPublished - Feb 2011

Keywords

  • Axonal transport
  • Cell death
  • Dysregulation
  • Motorneuron disease
  • Neurodegenerative disease

ASJC Scopus subject areas

  • Cell Biology

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