Decreased mRNA Expression of PGC-1α and PGC-1α-Regulated Factors in the SOD1^G93AALS Mouse Model and in Human Sporadic ALS

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by selective motoneuron loss. Although the cause of ALS is unknown, oxidative stress, inflammation, and mitochondrial dysfunction have been identified as important components of its pathogenesis. Peroxisome proliferator-activated receptor-γ coactivator 1α (PGC-1α) plays a central role in the regulation of mitochondrial...

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