Recent advances in mitochondrial aminoacyl-tRNA synthetases and disease

M Sissler, LE González-Serrano, E Westhof - Trends in molecular medicine, 2017 - cell.com
Dysfunctions in mitochondria–the powerhouses of the cell–lead to several human
pathologies. Because mitochondria integrate nuclear and mitochondrial genetic systems …

Fracture toughness of thermoelectric materials

G Li, Q An, B Duan, L Borgsmiller, M Al Malki… - Materials Science and …, 2021 - Elsevier
The engineering applications of thermoelectric (TE) devices require TE materials
possessing high TE performance and robust mechanical properties. Research on thermal …

Widespread translational control of fibrosis in the human heart by RNA-binding proteins

S Chothani, S Schäfer, E Adami, S Viswanathan… - Circulation, 2019 - Am Heart Assoc
Background: Fibrosis is a common pathology in many cardiac disorders and is driven by the
activation of resident fibroblasts. The global posttranscriptional mechanisms underlying …

Roles of aminoacyl-tRNA synthetases in immune regulation and immune diseases

A Nie, B Sun, Z Fu, D Yu - Cell death & disease, 2019 - nature.com
Aminoacyl-tRNA synthetases (ARSs) play a vital role in protein synthesis by linking amino
acids to their cognate transfer RNAs (tRNAs). This typical function has been well recognized …

RNA granule-clustered mitochondrial aminoacyl-tRNA synthetases form multiple complexes with the potential to fine-tune tRNA aminoacylation

GX Peng, XL Mao, Y Cao, SY Yao, QR Li… - Nucleic Acids …, 2022 - academic.oup.com
Mitochondrial RNA metabolism is suggested to occur in identified compartmentalized foci, ie
mitochondrial RNA granules (MRGs). Mitochondrial aminoacyl-tRNA synthetases (mito …

Rat models of human diseases and related phenotypes: a systematic inventory of the causative genes

C Szpirer - Journal of biomedical science, 2020 - Springer
The laboratory rat has been used for a long time as the model of choice in several
biomedical disciplines. Numerous inbred strains have been isolated, displaying a wide …

Mitochondrial aminoacyl-tRNA synthetase disorders: an emerging group of developmental disorders of myelination

AS Fine, CL Nemeth, ML Kaufman, A Fatemi - Journal of …, 2019 - Springer
Background The mitochondrial aminoacyl-tRNA synthetase proteins (mt-aaRSs) are a group
of nuclear-encoded enzymes that facilitate conjugation of each of the 20 amino acids to its …

The regulatory roles of aminoacyl-tRNA synthetase in cardiovascular disease

Y Zou, Y Yang, X Fu, X He, M Liu, T Zong, X Li… - … Therapy-Nucleic Acids, 2021 - cell.com
Aminoacyl-tRNA synthetases (ARSs) are widely found in organisms, which can activate
amino acids and make them bind to tRNA through ester bond to form the corresponding …

Mutations of the aminoacyl‐tRNA‐synthetases SARS and WARS2 are implicated in the etiology of autosomal recessive intellectual disability

L Musante, L Püttmann, K Kahrizi, M Garshasbi… - Human …, 2017 - Wiley Online Library
Intellectual disability (ID) is the hallmark of an extremely heterogeneous group of disorders
that comprises a wide variety of syndromic and non‐syndromic phenotypes. Here, we report …

The role of nuclear-encoded mitochondrial tRNA charging enzymes in human inherited disease

C Del Greco, A Antonellis - Genes, 2022 - mdpi.com
Aminoacyl-tRNA synthetases (ARSs) are highly conserved essential enzymes that charge
tRNA with cognate amino acids—the first step of protein synthesis. Of the 37 nuclear …