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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
MetabolomicsPhospholipid acyl chain diversity controls the tissue-specific assembly of mitochondrial cardiolipins

Cardiolipin (CL) is a phospholipid specific for mitochondrial membranes and crucial for many core tasks of this organelle. Its side chain configurations are tissue-specific, functionally important and generated via post-biosynthetic remodeling. However, this process lacks the necessary specificit...

2020-05-22 | MTBLS1446 | MetaboLights
MetabolomicsIntegrated Multi-Omics Mapping of Mitochondrial Dysfunction and Substrate Preference in Barth Syndrome Cardiac Tissue

Barth syndrome (BTHS) is a rare X-linked recessively inherited disorder caused by variants in the TAFAZZIN gene. The pathogenic variants lead to impaired conversion of monolysocardiolipin (MLCL) into mature phospholipid cardiolipin (CL). The accumulation of MLCL and mature CL deficiency is a diag...

2025-09-08 | MTBLS12953 | 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
MetabolomicsMitochondrial respiration controls neoangiogenesis during wound healing and tumour growth

The vasculature represents a highly plastic compartment, capable of switching from a quiescent to an active proliferative state during angiogenesis. Metabolic reprogramming in endothelial cells (ECs) thereby is crucial to cover the increasing cellular energy demand under growth conditions. Here w...

2020-06-29 | MTBLS1764 | 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
MetabolomicsFatty acid oxidation protects cancer cells from apoptosis through increasing mitochondrial membrane lipids

Overcoming resistance to chemotherapies remains a major unmet need for cancers such as triple negative breast cancer (TNBC). Therefore, mechanistic studies to provide insight for drug development are urgently needed to overcome TNBC therapy resistance. Recently, an important role of fatty acid β-...

2022-05-31 | MTBLS4709 | MetaboLights
MetabolomicsX chromosome dosage drives statin-induced dysglycemia and mitochondrial dysfunction

Statin drugs lower blood cholesterol levels for cardiovascular disease prevention. Women are more likely than men to experience adverse statin effects, particularly new-onset diabetes (NOD) and muscle weakness. Here we find that impaired glucose homeostasis and muscle weakness in statin-treated f...

2024-05-23 | MTBLS9677 | MetaboLights
MetabolomicsAntidiabetic and cardiovascular beneficial effects of a liver-localized mitochondrial uncoupler
Inducing mitochondrial uncoupling (mUncoupling) is an attractive therapeutic strategy for treating metabolic diseases because it leads to calorie-wasting by reducing the efficiency of oxidative phosphorylation (OXPHOS) in mitochondria. Here we report a safe mUncoupler, OPC-163493, which has unique p...
2019-05-24 | MTBLS836 | MetaboLights
MetabolomicsLipidomics of colon tissues

Pre-IBD is a transitional state between irritable bowel syndrome (IBS) and inflammatory bowel disease (IBD), in which chronic high-fat diet intake and antibiotic exposure significantly increase intestinal inflammatory susceptibility. However, the underlying mechanisms and effective therapeutic st...

2026-04-29 | MTBLS14399 | MetaboLights
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