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A human pathology-related mutation prevents import of an aminoacyl-tRNA synthetase into mitochondria.

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Messmer, Marie | Florentz, Catherine | Schwenzer, Hagen | Scheper, Gert C | van Der Knaap, Marjo S | Maréchal-Drouard, Laurence | Sissler, Marie

Edité par CCSD ; Portland Press -

International audience. Mutations in the nuclear gene coding for the mitochondrial aspartyl-tRNA synthetase, a key enzyme for mitochondrial translation, are correlated with leukoencephalopathy. A Ser45 to Gly45 mutation is located in the predicted targeting signal of the protein. We demonstrate in the present study, by in vivo and in vitro approaches, that this pathology-related mutation impairs the import process across mitochondrial membranes.

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