Wobble modification defect suppresses translational activity of tRNAs with MERRF and MELAS mutations

Takehiro Yasukawa, Tsutomu Suzuki, Shigeo Ohta, Kimitsuna Watanabe

Research output: Contribution to journalReview articlepeer-review

14 Citations (Scopus)

Abstract

By purifying mutant mitochondrial tRNAs, we were able to ascertain that post-transcriptional modification at the anticodon wobble uridine is absent in tRNALys with the 8344 MERRF mutation and in tRNALeu(UUR) with either the 3243 or 3271 MELAS mutation. Both the MERRF and MELAS mutant tRNAs substantially lost their translational ability, the extent of the loss in each mutant corresponding to the reduction in actual mitochondrial translational activity. Lack of the wobble modification deprived mutant tRNALys of interaction with the cognate codons. These features indicate that the modification defect plays a primary role in the molecular pathophysiology of these mitochondrial diseases.

Original languageEnglish
Pages (from-to)129-141
Number of pages13
JournalMitochondrion
Volume2
Issue number1-2
DOIs
Publication statusPublished - Jan 1 2002
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Molecular Medicine
  • Molecular Biology
  • Cell Biology

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