Ontology highlight
ABSTRACT:
SUBMITTER: Ruzzenente B
PROVIDER: S-EPMC3261557 | biostudies-literature | 2012 Jan
REPOSITORIES: biostudies-literature
Ruzzenente Benedetta B Metodiev Metodi D MD Wredenberg Anna A Bratic Ana A Park Chan Bae CB Cámara Yolanda Y Milenkovic Dusanka D Zickermann Volker V Wibom Rolf R Hultenby Kjell K Erdjument-Bromage Hediye H Tempst Paul P Brandt Ulrich U Stewart James B JB Gustafsson Claes M CM Larsson Nils-Göran NG
The EMBO journal 20111101 2
Regulation of mtDNA expression is critical for maintaining cellular energy homeostasis and may, in principle, occur at many different levels. The leucine-rich pentatricopeptide repeat containing (LRPPRC) protein regulates mitochondrial mRNA stability and an amino-acid substitution of this protein causes the French-Canadian type of Leigh syndrome (LSFC), a neurodegenerative disorder characterized by complex IV deficiency. We have generated conditional Lrpprc knockout mice and show here that the g ...[more]