Before a drug can enter clinical trials it generally must go through rigorous pre-clinical trials in the test tube and in animal models of disease. Therefore it is vital to have animal models that reflect the human disease to test these potential drugs on. There have been many mouse models of MND made using various mutations in the SOD1 gene associated with some familial forms. However, although these mice have given us a wealth of valuable information on how the disease may develop in humans, we have not seen this progress into much needed effective treatments. Some would argue that since new genetic mutations in human genes, such as that encoding TDP-43, have now been found to cause MND the time is now right to create new mouse models that may reflect sporadic MND more closely. It comes as no surprise then that a group of researchers from the Hope Center for Neurological Diseases, Washington, USA has made such a mouse. The researchers headed by Dr Robert H. Baloha, published their findings in the Proceedings of the National Academy of Sciences. This mouse model has some features similar to that of human MND, such as protein deposits and specific cell death of motor neurones. This is an exciting step forward and adds a powerful tool to the fight against MND.
Source: International MND research update -December 2009, Dr Justin Yerbury for MNDRIA