Dimerization of a pathogenic human mitochondrial tRNA

  title={Dimerization of a pathogenic human mitochondrial tRNA},
  author={Lisa M. Wittenhagen and Shana O. Kelley},
  journal={Nature Structural Biology},
Mutations of human mitochondrial transfer RNA (tRNA) are implicated in a variety of multisystemic diseases. The most prevalent pathogenic mitochondrial mutation is the A3243G substitution within the gene for tRNALeu(UUR). Here we describe the pronounced structural change promoted by this mutation. The A3243G mutation induces the formation of a tRNA dimer that strongly self-associates under physiological conditions. The dimerization interface in the mutant tRNA is a self-complementary… CONTINUE READING

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