Article
LRPPRC and SLIRP synergize to maintain sufficient and orderly mammalian mitochondrial translation.
Nucleic acids research - 14 Oct 2024
Rubalcava-Gracia Diana, Bubb Kristina, Levander Fredrik, Burr Stephen P, August Amelie V, Chinnery Patrick F, Koolmeister Camilla, Larsson Nils-Göran
Abstract excerpt
In mammals, the leucine-rich pentatricopeptide repeat protein (LRPPRC) and the stem-loop interacting RNA-binding protein (SLIRP) form a complex in the mitochondrial matrix that is required throughout the life cycle of most mitochondrial mRNAs. Although pathogenic mutations in the LRPPRC and SLIRP genes cause devastating human mitochondrial diseases, the in vivo function of the corresponding proteins is...
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