کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
5592880 | 1405044 | 2017 | 16 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Recent Advances in Mitochondrial Aminoacyl-tRNA Synthetases and Disease
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
پزشکی مولکولی
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چکیده انگلیسی
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.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Trends in Molecular Medicine - Volume 23, Issue 8, August 2017, Pages 693-708
Journal: Trends in Molecular Medicine - Volume 23, Issue 8, August 2017, Pages 693-708
نویسندگان
Marie Sissler, Ligia Elena González-Serrano, Eric Westhof,