Wobbler

Wobbler is a neurodegenerative disease model that arose in a laboratory mouse by chance. As it has a very valuable phenotype that mimics ALS and other motor neuron diseases, it was maintained by breeding. As it was a mutation that arose spontaneously, we currently have no information on where in the mouse genome the ALS-producing mutation is present. Currently investigators are trying to identify the location of the mutation in the mouse. Various therapies have been tested in this model and most of the drugs that worked in the model also worked in the genetic ALS model (SOD1G93A). Thus it appears that this is a good model for screening drugs.