Article
Wobble modification defect in tRNA disturbs codon-anticodon interaction in a mitochondrial disease.
The EMBO journal - 3 Sept 2001
Yasukawa T, Suzuki T, Ishii N, Ohta S, Watanabe K
Abstract excerpt
We previously showed that in mitochondrial tRNA(Lys) with an A8344G mutation responsible for myoclonus epilepsy associated with ragged-red fibers (MERRF), a subgroup of mitochondrial encephalomyopathic diseases, the normally modified wobble base (a 2-thiouridine derivative) remains unmodified. Since wobble base modifications are essential for translational efficiency and accuracy, we used mitochondrial components...
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