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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
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
MetabolomicsCharacterization of the heat-stable proteome during seed germination in Arabidopsis with special focus on LEA proteins
During seed germination, desiccation tolerance is lost in the radicle with progressing radicle protrusion and seedling establishment. This process is accompanied by comprehensive changes of the metabolome and proteome. Germination of Arabidopsis seeds was investigated over 72 h with special focus on...
2021-07-29 | MTBLS2980 | MetaboLights
MetabolomicsMembrane lipid as cofactor of cargo uptake and release by box-like lipid transfer proteins
Lipid transfer proteins (LTPs) shuttle their cargo lipids between cellular membranes. LTPs facilitate the costly extraction of a lipid from a donor membrane, and its delivery into the acceptor membrane, but the mechanisms remain poorly understood. We combined extensive molecular dynamics simulations...
2026-10-06 | MTBLS15933 | 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
MetabolomicsSIN-3 transcriptional coregulator maintains mitochondrial homeostasis and polyamine flux
Mitochondrial function relies on the coordinated transcription of mitochondrial and nuclear genomes to assemble respiratory chain complexes. Across species, the SIN3 coregulator influences mitochondrial functions, but how its loss impacts mitochondrial homeostasis and metabolism in the context of a ...
2024-04-01 | MTBLS8439 | MetaboLights
MetabolomicsPRODH regultes naive pluripotency by influencing mitochondrial oxidative phosphorylation (Untargeted metabolomics)

Naïve human embryonic stem cells (hESCs) that resemble the pre-implantation epiblasts are fueled by a combination of aerobic glycolysis and oxidative phosphorylation, but their mitochondrial regulators are poorly understood. Here we report that, proline dehydrogenase (PRODH), a mitochondria-local...

2024-02-12 | MTBLS7840 | MetaboLights
MetabolomicsPRODH regultes naive pluripotency by influencing mitochondrial oxidative phosphorylation (Targeted metabolomics)

Naïve human embryonic stem cells (hESCs) that resemble the pre-implantation epiblasts are fueled by a combination of aerobic glycolysis and oxidative phosphorylation, but their mitochondrial regulators are poorly understood. Here we report that, proline dehydrogenase (PRODH), a mitochondria-local...

2024-02-12 | MTBLS7832 | 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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