Pathogenesis of bacterial infections and immunity

@article{,

title = {Proteomic consequences of a human mitochondrial tRNA mutation beyond the frame of mitochondrial translation},

author = {P Tryoen-Toth and S Richert and B Sohm and M Mine and C Marsac and A Van Dorsselaer and E Leize and C Florentz},

url = {http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12714596},

isbn = {12714596},

year  = {2003},

date = {2003-01-01},

journal = {J Biol Chem},

volume = {278},

number = {27},

pages = {24314-24323},

abstract = {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 approach. Here a quantitative analysis of several hundred silver-stained spots separated by two-dimensional gel electrophoresis was performed in the specific case of a couple of mitochondria, containing or not mutation A8344G in the gene for mitochondrial tRNALys, correlated with MERRF syndrome. Computer-assisted analysis allowed us to detect 38 spots with significant quantitative variations, of which 20 could be assigned by mass spectrometry. These include nuclear encoded proteins located in mitochondria such as respiratory chain subunits, metabolic enzymes, a protein of the mitochondrial translation machinery, and cytosolic contaminants. Furthermore, Western blotting combined with mass spectrometry revealed the occurrence of numerous isoforms of pyruvate dehydrogenase subunits, with subtle changes in post-translational modifications. This comparative proteomic approach gives the first insight for nuclear encoded proteins that undergo the largest quantitative changes, and pinpoints new potential molecular partners involved in the cascade of events that connect genotype to phenotype.},

note = {0021-9258 

Journal Article},

keywords = {Cultured, FLORENTZ, Genetic  Tumor Cells, Human  Mitochondria/genetics  *Mutation  Nuclear Proteins/*genetics  Proteomics  RNA/*genetics  RNA, Non-U.S. Gov't  Translation, Transfer/*genetics  Structure-Activity Relationship  Support, Unité ARN},

pubstate = {published},

tppubtype = {article}

}