Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
2051436 | FEBS Letters | 2005 | 5 Pages |
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 [Ïm5(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.
Related Topics
Life Sciences
Agricultural and Biological Sciences
Plant Science
Authors
Takehiro Yasukawa, Yohei Kirino, Norie Ishii, Ian J. Holt, Howard T. Jacobs, Takao Makifuchi, Nobuyoshi Fukuhara, Shigeo Ohta, Tsutomu Suzuki, Kimitsuna Watanabe,