Neurodegenerative diseases are incurable and debilitating conditions that result in progressive degeneration and death of nerve cells. There is an urgent need for effective neuroprotective therapies for neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS).

Data from disease model systems and from human biosamples provide strong evidence for a role of redox imbalance, inflammation, mitochondrial dysfunction and altered proteostasis including autophagy and mitophagy, as four key main drivers in the pathobiology of neurodegenerative diseases. Compounds which activate the nuclear factor erythroid 2-related factor 2 (NRF2) and heat shock factor 1 (HSF1) pathways have beneficial effects on all four of these key main drivers of neurodegeneration.