Table 4

 Cell specific features of motor neurones predisposing to neurodegeneration

• Cell size and axonal length
• High metabolic rate
• ? Specific features of motor neurone mitochondria
• Cytoskeleton, neurofilament content and reliance on efficient intracellular transport system
• Characteristic profile of cell surface glutamate receptors, with high relative expression of calcium permeable AMPA receptors, lacking the GluR2 subunit
• High expression of glutamate transporters in the vicinity of vulnerable motor neurone groups
• Low expression of specific calcium binding proteins
• High expression of SOD1 protein
• High threshold for mounting a heat shock response/upregulation of chaperone proteins