ALS is caused by strains of SOD1 prions
The Swedish Brain Foundation/Swedish Research Council
There is no effective therapy for ALS. More than 15 clinical trials using different drugs have been conducted since the turn of the century, and all have failed. The lack of understanding of the underlying disease mechanisms have no doubt contributed to this miserable result. The ALS model in which hSOD1 variants are expressed in rodents, is the
neurodegenerative disease model which most faithfully recapitulates the modeled disease in humans. In this model, we here show that the basic disease mechanism is an exponential templated replication of hSOD1 prion strains. This novel insight promises to lead to development of both novel rational therapies and novel diagnostics.