Article
Proteomic consequences of a human mitochondrial tRNA mutation beyond the frame of mitochondrial translation.
The Journal of biological chemistry - 4 Jul 2003
Tryoen-Tóth Petra, Richert Sophie, Sohm Bénédicte, Mine Manuele, Marsac Cécile, Van Dorsselaer Alain, Leize Emmanuelle, Florentz Catherine
Abstract excerpt
Numerous severe neurodegenerative and neuromuscular disorders, characterized biochemically by strong perturbations in energy metabolism, are correlated with single point mutations in mitochondrial genes coding for transfer RNAs. Initial comparative proteomics performed on wild-type and Myoclonic Epilepsy and Ragged Red Fibers (MERRF) mitochondria from sibling human cybrid cell lines revealed the potential of this...
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