Biologie Digitale de l’ARN

@article{,

title = {Recent Advances in Mitochondrial Aminoacyl-tRNA Synthetases and Disease},

author = {M Sissler and L E González-Serrano and E Westhof},

url = {https://www.ncbi.nlm.nih.gov/pubmed/28716624?dopt=Abstract},

doi = {10.1016/j.molmed.2017.06.002},

isbn = {28716624},

year  = {2017},

date = {2017-01-01},

journal = {Trends Mol Med},

volume = {23},

number = {8},

pages = {693-708},

abstract = {Dysfunctions in mitochondria - the powerhouses of the cell - lead to several human pathologies. Because mitochondria integrate nuclear and mitochondrial genetic systems, they are richly intertwined with cellular activities. The nucleus-encoded mitochondrial aminoacyl-tRNA synthetases (mt-aaRSs) are key components of the mitochondrial translation apparatus. Mutations in these enzymes predominantly affect the central nervous system (CNS) but also target other organs. Comparable mutations in mt-aaRSs can lead to vastly diverse diseases, occurring at different stages in life, and within different tissues; this represents a confounding issue. With newer information available, we propose that the pleiotropy and tissue-specificity of mt-aaRS-associated diseases result from the molecular integration of mitochondrial translation events within the cell; namely, through specific crosstalk between the cellular program and the energy demands of the cell. We place particular focus on neuronal cells.},

keywords = {aminoacyl-tRNA synthetase central nervous system mitochondrial disease mitochondrial translation moonlighting proteins unfolded protein response, SISSLER, Unité ARN, WESTHOF},

pubstate = {published},

tppubtype = {article}

}