A protein involved in spinal muscular atrophy, called ‘survival motor neurone’ (SMN), has previously been shown to be low in sporadic MND patients. A research team led by Dr Bradley Turner from the Howard Florey Institute in Melbourne has studied the role of the loss of the SMN protein in mice with mutant SOD1 associated MND. They found that the loss of SMN resulted in the shortening of MND mice lifespan.
Amazingly the function of this protein is similar to that of FUS and TDP¬43 in that it also processes and transports messenger RNA. It is very interesting to note that the results also show that mutant SOD1 induces mRNA processing dysfunction. Thus, this important work suggests that SOD1 along with all other familial MND gene mutations found so far have some effect on a common process. In additions, the work suggests that supplementation of SMN might be a viable treatment for MND patients.
Source: International MND research update – June 2009, Dr Justin Yerbury for MNDRIA