Receptor alterations associated with spinal motoneuron degeneration in bovine Akabane disease.

Akabane disease in cattle is characterized by congenital abnormalities including arthrogryposis, which is characterized by a depletion of spinal ventral horn motoneurons, a loss of axons, and depletion of myelin in the lateral and ventral tracts. These neuropathological changes produced major reductions (70-80%) in the density of muscarinic cholinergic, glycine/strychnine, and central-type benzodiazepine receptors in the ventral horn motor nuclei. The density of peripheral-type benzodiazepine receptors and adenosine A1 receptors was dramatically increased (250-300%) in the lateral and ventral spinal columns, reflecting the proliferation of glial cells. Bovine Akabane disease represents a useful model for assessing the processes and consequences of neuronal degeneration and demyelination and has implications for human diseases such as amyotrophic lateral sclerosis.