Axonal transport

Axonal transport has become an important new area of ALS research. Genetics, and some mutant ALS genes, have incriminated the process of axonal transport, which is pathological in both sporadic and familial ALS.

Transport of materials along the length of the motor neuron is an important cellular process that may contribute to the damage seen in ALS patients. In healthy individuals, neurons move cellular materials along their axons, which keep nerve cell messages flowing and maintain the health of the overall nerve cell. However, motor neurons can be vulnerable to genetic or cellular defects that impede axonal transport. Studies of the proteins within nerve fibers point to the possible role of these axon proteins in ALS. Selective death of motor neurons can lead to progressive muscle weakness in ALS patients.

New findings indicate that in some cases of sporadic ALS (with no mutations in the SOD1 gene or protein), the SOD1 protein gets misfolded and can directly inhibit axonal transport.