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- Title
Wobble modification deficiency in mutant tRNAs in patients with mitochondrial diseases
- Authors
Yasukawa, Takehiro; Kirino, Yohei; Ishii, Norie; Holt, Ian J.; Jacobs, Howard T.; Makifuchi, Takao; Fukuhara, Nobuyoshi; Ohta, Shigeo; Suzuki, Tsutomu; Watanabe, Kimitsuna
- Abstract
Abstract: Point mutations in mitochondrial (mt) tRNA genes are associated with a variety of human mitochondrial diseases. We have shown previously that mt tRNALeu(UUR) with a MELAS A3243G mutation and mt tRNALys with a MERRF A8344G mutation derived from HeLa background cybrid cells are deficient in normal taurine-containing modifications [τm5(s2)U; 5-taurinomethyl-(2-thio)uridine] at the anticodon wobble position in both cases. The wobble modification deficiency results in defective translation. We report here wobble modification deficiencies of mutant mt tRNAs from cybrid cells with different nuclear backgrounds, as well as from patient tissues. These findings demonstrate the generality of the wobble modification deficiency in mutant tRNAs in MELAS and MERRF.
- Subjects
TRANSFER RNA; AMINOACYL-tRNA; ORGANS (Anatomy); PRESERVATION of organs, tissues, etc.
- Publication
FEBS Letters, 2005, Vol 579, Issue 13, p2948
- ISSN
0014-5793
- Publication type
Article
- DOI
10.1016/j.febslet.2005.04.038