Minimal effects of (PGC-1) overexpression in a mitochondrial disease model.

Biol Open

Faculty of Medicine and Health Technology, FI-33014 Tampere University, Finland

Published: July 2019

PGC-1α and its homologues have been proposed to act as master regulators of mitochondrial biogenesis in animals. Most relevant studies have been conducted in mammals, where interpretation is complicated by the fact that there are three partially redundant members of the gene family. In , only a single PGC-1 homologue, (), is present in the genome. Here, we analyzed the effects of overexpression on phenotype and on gene expression in , a recessive bang-sensitive mutant with a global defect in oxidative phosphorylation, resulting from a deficiency of mitochondrial protein synthesis. In contrast to previous reports, we found that substantial overexpression of throughout development had only minimal effects on the mutant phenotype. Copy number of mtDNA was unaltered and overexpression produced no systematic effects on a representative set of transcripts related to mitochondrial OXPHOS and other metabolic enzymes, although these were influenced by sex and genetic background. This study provides no support to the concept of Spargel as a global regulator of mitochondrial biogenesis, at least in the context of the model.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6679408PMC
http://dx.doi.org/10.1242/bio.042135DOI Listing

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