Axon pathology as a therapeutic target in Huntingdon's disease

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Huntington's disease is a devastating, progressive deterioration in movement control accompanied by serious psychiatric defects that is strictly inherited. Each generation tends to develop the disease earlier than the last. The gene has been identified and a range of animal models have been generated. The disease mechanism is partially understood but much more needs to be done to move towards therapy. One of the major remaining questions involves the roles of axons, the long wire-like strands connecting each nerve cell with the next. Without these wires, nerve cells cannot function and parts of the nervous system stop working. One problem with studying axons is that they are so long, so it is not easy to see them microscopically in their entirety. We have developed new imaging methods to overcome these problems that we have already applied to mouse models of Alzheimer's disease. Here we will apply these same methods to Huntington's disease to determine where axon pathology fits into the sequence of events leading to this disease.

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