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Article

Human mitochondrial tRNA quality control in health and disease: a channelling mechanism?

RNA biology - 1 Jan 2012

Belostotsky Ruth, Frishberg Yaacov, Entelis Nina

Abstract excerpt

Mutations in human mitochondrial tRNA genes are associated with a number of multisystemic disorders. These single nucleotide substitutions in various domains of tRNA molecules may affect different steps of tRNA biogenesis. Often, the prominent decrease of aminoacylation and/or steady-state levels of affected mitochondrial tRNA have been demonstrated in patients' tissues and in cultured cells. Similar effect has...

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PMID: 22258151
DOI: 10.4161/rna.9.1.18009

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