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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
MetabolomicsMitochondrial NAD+ Deficiency in Vascular Smooth Muscle Impairs Collagen III Turnover to Trigger Aortic Disease

Aortic disease poses a significant mortality risk in adults, yet many of its underlying factors remain undiscovered. In this study, we identify mitochondrial NAD⁺ deficiency as a causal factor. Metabolomics analysis of 73 surgical aortic specimens revealed impaired NAD⁺ salvage and mitochondrial ...

2024-12-04 | MTBLS8062 | 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
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
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 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
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
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
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
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