Defective axonal transport in motor neuron disease

J Neurosci Res. 2007 Sep;85(12):2557-66. doi: 10.1002/jnr.21188.

Abstract

Several recent studies have highlighted the role of axonal transport in the pathogenesis of motor neuron diseases. Mutations in genes that control microtubule regulation and dynamics have been shown to cause motor neuron degeneration in mice and in a form of human motor neuron disease. In addition, mutations in the molecular motors dynein and kinesins and several proteins associated with the membranes of intracellular vesicles that undergo transport cause motor neuron degeneration in humans and mice. Paradoxically, evidence from studies on the legs at odd angles (Loa) mouse and a transgenic mouse model for human motor neuron disease suggest that partial limitation of the function of dynein may in fact lead to improved axonal transport in the transgenic mouse, leading to delayed disease onset and increased life span.

Publication types

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

MeSH terms

  • Animals
  • Axonal Transport / physiology*
  • Axons / metabolism*
  • Humans
  • Molecular Motor Proteins / genetics
  • Molecular Motor Proteins / metabolism
  • Motor Neuron Disease / pathology*
  • Motor Neuron Disease / physiopathology*
  • Mutation
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism

Substances

  • Molecular Motor Proteins
  • Nerve Tissue Proteins