Article
Fragile T-stem in disease-associated human mitochondrial tRNA sensitizes structure to local and distant mutations.
The Journal of biological chemistry - 6 Apr 2001
Kelley S O, Steinberg S V, Schimmel P
Abstract excerpt
Mutations in human mitochondrial isoleucine tRNA (hs mt tRNA(Ile)) are associated with cardiomyopathy and opthalmoplegia. A recent study showed that opthalmoplegia-related mutations gave rise to severe decreases in aminoacylation efficiencies and that the defective mutant tRNAs were effective inhibitors of aminoacylation of the wild-type substrate. The results suggested that the effectiveness of the mutations was...
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