title: Reduced expression of frataxin extends the lifespan of Caenorhabditis elegans.
abstract: Defects in the expression of the mitochondrial protein frataxin cause Friedreich's ataxia, an hereditary neurodegenerative syndrome characterized by progressive ataxia and associated with reduced life expectancy in humans. Homozygous inactivation of the frataxin gene results in embryonic lethality in mice, suggesting that frataxin is required for organismic survival. Intriguingly, the inactivation of many mitochondrial genes in the nematode Caenorhabditis elegans by RNAi extends lifespan. We therefore investigated whether inactivation of frataxin by RNAi-mediated suppression of the frataxin homolog gene (frh-1) would also prolong lifespan in the nematode. Frataxin-deficient animals have a small body size, reduced fertility and altered responses to oxidative stress. Importantly, frataxin suppression by RNAi significantly extends lifespan in C. elegans.
authors.affiliation: Institute for Behavioral Genetics, University of Colorado at Boulder, Box 447 Boulder, CO 80309-0447 USA.
authors.forename: Natascia
authors.initials: N
authors.lastname: Ventura
authors.forename: Shane
authors.initials: S
authors.lastname: Rea
authors.forename: Samuel T
authors.initials: ST
authors.lastname: Henderson
authors.forename: Ivano
authors.initials: I
authors.lastname: Condo
authors.forename: Thomas E
authors.initials: TE
authors.lastname: Johnson
authors.forename: Roberto
authors.initials: R
authors.lastname: Testi
mesh_terms: D000818:Animals; D049628:Body Size; D017173:Caenorhabditis elegans / Q000235:genetics / Q000502:physiology*; D005298:Fertility; D033862:Iron-Binding Proteins / Q000096:biosynthesis* / Q000235:genetics; D008136:Longevity; D034622:RNA Interference; D000096923:Frataxin
chemical_list: D033862:Iron-Binding Proteins; D000096923:Frataxin