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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
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
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
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 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
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
MetabolomicsATAD3A has a scaffolding role regulating mitochondria inner membrane structure and protein assembly

The ATPase Family AAA Domain Containing 3A (ATAD3A), is a mitochondrial inner membrane protein conserved in metazoans. ATAD3A has been associated with several mitochondrial functions, including nucleoid organization, cholesterol metabolism, and mitochondrial translation. To address its primary ro...

2022-01-14 | MTBLS3786 | MetaboLights
TranscriptomicsThe human mitochondrial transcriptome
The human mitochondrial genome comprises a distinct genetic system transcribed as precursor polycistronic transcripts that are subsequently cleaved to generate individual mRNAs, tRNAs and rRNAs. Here we provide a comprehensive analysis of the human mitochondrial transcriptome across multiple cell li...
ORGANISM(S): Homo sapiens 
2011-07-20 | E-GEOD-30772 | biostudies-arrayexpress
MetabolomicsThermal Profiling Maps Multilayered Organization of the Mitochondrial Proteome

Mitochondria execute essential metabolic, biosynthetic, and signaling functions, yet the regulatory principles governing their proteome remain incompletely understood. Here, we develop a thermal proteome profiling (TPP) workflow applied directly to purified mitochondria, enabling systematic analy...

2025-12-08 | MTBLS7386 | MetaboLights
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