Peroxisome proliferator-activated receptor gamma coactivator-1alpha and peroxisome proliferator-activated receptor delta expression in a model of amyotrophic lateral schlerosis.

Amyotrophic lateral sclerosis is a fatal paralytic disorder characterized by premature motor neuron death; a hypermetabolic state has also been observed. Western blotting was used to characterize the expression of PGC-1alpha and PPAR-delta, two metabolic regulators, in tissue of G85R ALS mice and wild-type littermates at different development time points. Initial results indicate that PGC-1 alpha may be expressed at higher levels in skeletal muscle of young ALS mice, suggesting PGC-1alpha's potential involvement in the hypermetabolism evident in ALS.