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MetabolomicsRibosomal mistranslation leads to silencing of the unfolded protein response and increased mitochondrial biogenesis

Translation fidelity is the limiting factor in the accuracy of gene expression. With an estimated frequency of 10-4, errors in mRNA decoding occur in a mostly stochastic manner. Little is known about the response of higher eukaryotes to chronic loss of ribosomal accuracy as per an increase in the...

2019-09-17 | MTBLS106 | MetaboLights
MetabolomicsTargeted degradation of the α-ketoglutarate dehydrogenase mediated by a mitochondrial DNAJC protein regulates metabolism

Mitochondrial heat shock proteins and co-chaperones play essential roles in maintaining proteostasis. They are renowned for regulating unfolded proteins, usually without specific target preferences. In this study, we identified T cell activation inhibitor, mitochondria (TCAIM), as a novel DNAJC-t...

2024-12-03 | MTBLS10440 | MetaboLights
MetabolomicsProtein-metabolite interactomics of carbohydrate metabolism reveal regulation of lactate dehydrogenase

Metabolic networks are interconnected and influence diverse cellular processes. The protein-metabolite interactions that mediate these networks are frequently low affinity and challenging to systematically discover. We developed mass spectrometry integrated with equilibrium dialysis for the disco...

2023-08-07 | MTBLS7237 | MetaboLights
MetabolomicsMitochondrial glutamine import sustains mitochondrial translation independent of glutaminolysis in cancer_GlnFlux_untargeted metabolites

Oxidative phosphorylation (OXPHOS) fulfills energy metabolism and biosynthesis through tricarboxylic acid (TCA) cycle and an intact electron transport chain (ETC). Mitochondrial glutamine import (MGI) replenishes TCA cycle through glutaminolysis, but its broader roles in cancer remains unclear. H...

2025-09-29 | MTBLS13074 | MetaboLights
MetabolomicsMitochondrial glutamine import sustains mitochondrial translation independent of glutaminolysis in cancer_targeted metabolites

Oxidative phosphorylation (OXPHOS) fulfills energy metabolism and biosynthesis through tricarboxylic acid (TCA) cycle and an intact electron transport chain (ETC). Mitochondrial glutamine import (MGI) replenishes TCA cycle through glutaminolysis, but its broader roles in cancer remains unclear. H...

2025-09-29 | MTBLS13076 | MetaboLights
MetabolomicsDeficient Endoplasmic Reticulum-Mitochondrial Phosphatidylserine Transfer Causes Liver Disease
Non-alcoholic fatty liver is the most common liver disease worldwide. Here, we show that the mitochondrial protein mitofusin 2 (Mfn2) protects against liver disease. Reduced Mfn2 expression was detected in liver biopsies from patients with non-alcoholic steatohepatitis (NASH). Moreover, reduced Mfn2...
2018-01-25 | MTBLS600 | MetaboLights
MetabolomicsMulti‐omics identify xanthine as a pro‐survival metabolite for nematodes with mitochondrial dysfunction
Aberrant mitochondrial function contributes to the pathogenesis of various metabolic and chronic disorders. Inhibition of insulin/IGF-1 signaling (IIS) represents a promising avenue for the treatment of mitochondrial diseases, although many of the molecular mechanisms underlying this beneficial effe...
2019-02-28 | MTBLS794 | MetaboLights
MetabolomicsMitochondrial metabolism sustains DNMT3A-R882-mutant clonal haematopoiesis
Somatic DNMT3A R882 codon mutations drive the most common form of clonal haematopoiesis (CH) and are associated with increased acute myeloid leukaemia (AML) risk1,2. Preventing expansion of DNMT3A-R882-mutant haematopoietic stem/progenitor cells (HSPCs) may therefore avert progression to AML. To ide...
2025-02-06 | MTBLS12201 | MetaboLights
MetabolomicsMitochondrial glutamine import sustains mitochondrial translation independent of glutaminolysis in cancer_A549 cell culture_untargeted metabolites_U_13gln/glc

Oxidative phosphorylation (OXPHOS) fulfills energy metabolism and biosynthesis through tricarboxylic acid (TCA) cycle and an intact electron transport chain (ETC). Mitochondrial glutamine import (MGI) replenishes TCA cycle through glutaminolysis, but its broader roles in cancer remains unclear. H...

2025-09-29 | MTBLS13075 | MetaboLights
MetabolomicsMitochondrial Transplantation Ameliorates Doxorubicin-Induced Cardiac Dysfunction via Activating Glutamine Metabolism
Doxorubicin is a wildly used effective anticancer agent. However, doxorubicin use is also related to cardiotoxic side effect in some patients. Mitochondrial damage has been shown to be one of the pathogeneses of doxorubicin-induced myocardial injury. In this study, we test the hypothesis that mitoch...
2025-03-13 | MTBLS7989 | MetaboLights
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