Reduced mRNA and protein expression of the transcriptional co-activator PGC-1alpha in post-mortem tissue of ALS patients and in the G93A transgenic ALS mouse model

Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disorder with an average survival of 3–5 years after the onset of symptoms. It is characterized by a rapidly progressive paralysis due to the degeneration of motor neurons in primary motor cortex, brain stem and spinal cord. About 90% of cases occur sporadically and 10% are familial. Among the familial cases, 10–20% can be attributed to point mutations in the gene coding for the antioxidant enzyme Cu/Zn superoxide dismutase (SOD1) (Rosen et al., 1993). This discovery led to the generation of mouse models overexpressing common human S…