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MetabolomicsCardiac disruption of SDHAF4-mediated mitochondrial complex II assembly promotes dilated cardiomyopathy
Succinate dehydrogenase, which is known as mitochondrial complex II, has proven to be a fascinating machinery, attracting renewed and increased interest in its involvement in human diseases. Herein, we find that succinate dehydrogenase assembly factor 4 (SDHAF4) is downregulated in cardiac muscle in...
2022-08-12 | MTBLS4579 | MetaboLights
MetabolomicsFate and PPARγ and STATs-driven effects of the mitochondrial complex I inhibitor tebufenpyrad in liver cells revealed with multi-omics.
The biological effects of the pesticide and mitochondrial complex I inhibitor tebufenpyrad (TEBU) on liver cells were investigated by combining proteomics and metabolomics. Both cell culture media and cellular lysates were analyzed in dose-response and kinetic experiments on the HepaRG cell line. Re...
2024-02-07 | MTBLS4063 | MetaboLights
MetabolomicsSARS-CoV-2 ORF7a Drives Mitochondrial Dysfunction via PDK4 Activation and Complex I Inhibition

SARS-CoV-2 reprograms host metabolism to promote viral replication and evade immune responses. While infection is known to impair mitochondrial function and enhance glycolysis, the role of viral accessory proteins in these alterations remains unclear. Here, we investigate the metabolic impact of ...

2026-07-09 | MTBLS13338 | 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
MetabolomicsABHD11/ROLIP maintains the TCA cycle by preserving functional lipoylation of the 2-oxoglutarate dehydrogenase complex
2-oxoglutarate (2-OG) relates mitochondrial metabolism to cell function: conversion of 2-OG to succinyl-CoA by the 2-OG dehydrogenase complex (OGDHc) is rate-limiting for the Tricarboxylic Acid (TCA) cycle, but 2-OG is also required for oxygen sensitive enzymes (2-OG dependent dioxygenases) that hav...
2020-08-10 | MTBLS1875 | MetaboLights
MetabolomicsSelective galactose culture condition reveals distinct metabolic signatures in pyruvate dehydrogenase and complex I deficient human skin fibroblasts

INTRODUCTION: A decline in mitochondrial function represents a key factor of a large number of inborn errors of metabolism, which lead to an extremely heterogeneous group of disorders.

OBJECTIVES: To gain insight into the biochemical consequences of mitocho...

2021-10-06 | MTBLS761 | MetaboLights
MetabolomicsMolecular structural diversity of mitochondrial cardiolipins
Current strategies used to quantitatively describe the biological diversity of lipids by mass spectrometry are often limited in assessing the exact structural variability of individual molecular species in detail. A major challenge is represented by the extensive isobaric overlap present among lipid...
2021-01-11 | MTBLS636 | MetaboLights
MetabolomicsDefective mitochondrial COX1 translation due to loss of COX14 function triggers ROS-induced inflammation in liver
Mitochondrial oxidative phosphorylation (OXPHOS) fuels cellular ATP demands. OXPHOS defects lead to severe human disorders with unexplained tissue specific pathologies. Mitochondrial gene expression is essential for OXPHOS biogenesis since core subunits of the complexes are mitochondrial-encoded. CO...
2024-07-19 | MTBLS9823 | MetaboLights
MetabolomicsPathological glutaminolysis induces mitochondrial injury via OPA1 glutamylation in renal fibrosis

The promotion of repair in proximal renal tubular epithelial cells (PTEC) or the prevention of repair failure has been suggested as a beneficial strategy for mitigating the progression of chronic kidney disease (CKD). However, effective interventions to halt CKD progression and subsequent renal f...

2025-10-23 | MTBLS13148 | MetaboLights
MetabolomicsEpistasis-driven identification of SLC25A51 as a regulator of human mitochondrial NAD import
About a thousand genes in the human genome encode for membrane transporters. Among these, several solute carrier proteins (SLCs), representing the largest group of transporters, are still orphan and lack functional characterization. We reasoned that assessing genetic interactions among SLCs may be a...
2021-03-29 | MTBLS2205 | MetaboLights
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