Abstract:
:The pathogenetic mechanism of the most extensively investigated A3243G mutated tRNALeu(UUR) gene, which causes the MELAS encephalomyopathy, maternally inherited diabetes, or chronic progressive external ophlthalmoplegia, is still unresolved, despite the numerous investigations on the topic. Previous evidences presented in published work suggested that the mitochondrial DNA harboring A3243G mutation result decreases in the rates of mitochondrial protein synthesis. To search for differences in aminoacylation of mitochondrial DNA-encoded wild-type and mutant human tRNALeu(UUR), we have expressed and purified the two kinds of tRNAsLeu(UUR), and have expressed human mitochondrial leucyl-tRNA synthetase for in vitro assays of aminoacylation of wild-type and mutant human tRNALeu(UUR). The results indicate human mitochondrial tRNALeu(UUR) gene A3243G point mutant can remarkably reduce its aminoacylation, suggesting it could be one of the mechanisms that the mutation can produce in such clinical phenotypes.
journal_name
IUBMB Lifejournal_title
IUBMB lifeauthors
Wang ZC,Wang XM,Jin YX,Jiao BH,Xu F,Miao MY,Zhu KJdoi
10.1080/1521654031000110190keywords:
subject
Has Abstractpub_date
2003-03-01 00:00:00pages
139-44issue
3eissn
1521-6543issn
1521-6551journal_volume
55pub_type
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