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MetabolomicsGenetic Ablation of Sfxn5 Induces Mitochondrial Dysfunction and Precipitates Lethal Metabolic Crisis in Mice
Mitochondrial disorders frequently manifest with life-threatening hepatic metabolic crises. Using a global Sfxn5-knockout mouse model, we investigated the systemic consequences of disrupting this mitochondrial inner-membrane transporter through integrated biochemical, metabolomic, histological, and ...
2026-05-30 | MTBLS14628 | MetaboLights
TranscriptomicsGene expression analysis in 13 patients with mitochondrial ATP synthase deficiency (Agilent)
Defects of mitochondrial functions lead in humans to vast array of usually multisystemic pathologies and several hundreds of diseases resulting from various defects of mitochondria biogenesis and maintenance, defects of respiratory chain complexes (OXPHOS) or defects of individual mitochondrial prot...
ORGANISM(S): Homo sapiens 
2008-08-01 | E-GEOD-10956 | biostudies-arrayexpress
GenomicsABHD11-mediated mtDNA transcription restored mitochondrial function to attenuate cardiomyocyte ferroptosis after myocardial infarction
ABHD11-mediated mtDNA transcription restored mitochondrial function to attenuate cardiomyocyte ferroptosis after myocardial infarction
PRJNA1433500 | ENA
ProteomicsStable nuclear expression of ATP8 and ATP6 genes rescues a mtDNA Complex V null mutant
We explore the possibility of re-engineering mitochondrial genes and expressing them from the nucleus as an approach to rescue defects arising from mitochondrial DNA mutations. We have used a patient cybrid cell line with a single point mutation in the overlap region of the ATP8 and ATP6 genes of th...
ORGANISM(S): Homo Sapiens (human) 
2016-08-12 | MSV000080037 | MassIVE
TranscriptomicsMesenchymal Stem Cells Transfer Mitochondria to the Cells with Virtually Absent Mitochondrial Function but Not to the Cells Harboring Pathogenic Mitochondrial DNA Mutations
It has been reported that human mesenchymal stem cells (MSCs) can transfer mitochondria to the cells with severely compromised mitochondrial function. We tested whether MSCs transfer mitochondria to the cells under several different conditions of mitochondrial dysfunction, including human pathogenic...
ORGANISM(S): Homo sapiens 
2011-11-22 | E-GEOD-33831 | biostudies-arrayexpress
GenomicsControl of microRNA expression by mitochondrial function
Control of microRNA expression by mitochondrial function
PRJNA412221 | ENA
ProteomicsMitochondrial IntegrityMitochondrial Integrity Modulates mTOR Signaling and Podocyte Function Modulates mTOR Signaling and Podocyte Function
Mitochondrial dysfunction has emerged as a key contributor to the pathogenesis of steroid-resistant nephrotic syndrome (SRNS) and genetic focal-segmental glomerulosclerosis (FSGS). This study explores the role of mitochondrial integrity in podocyte biology, focusing on the impact of OMA1, a critical...
ORGANISM(S): Mus musculus (Mouse) 
2025-11-26 | PXD071171 | Pride
Mitochondria Glycolysis 
TranscriptomicsClosing the mitochondrial permeability transition pore in hiPSC-derived endothelial cells induces glycocalyx formation and functional maturation.
Human induced pluripotent stem cells (hiPSCs) are used to study organogenesis and model disease as well as being developed for regenerative medicine. Endothelial cells are among the many cell types differentiated from hiPSC, but their maturation and stabilization fall short of that in adult endothel...
ORGANISM(S): Homo sapiens 
2019-12-14 | E-MTAB-8392 | biostudies-arrayexpress
TranscriptomicsHigh mitochondrial respiration and glycolytic capacity represent a metabolic phenotype of human tolerogenic dendritic cells
Human monocyte-derived dendritic cells (moDCs) have been used as an in vitro model for studying tolerance and immunity. However, the underlying metabolic states of tolerogenic (dexamethasone and vitamin D3-treated), immature and immunogenic (mature, LPS-treated) moDCs have not been completely charac...
ORGANISM(S): Homo sapiens 
2015-05-08 | E-GEOD-52894 | biostudies-arrayexpress
UnknownSS-31 alleviated nociceptive responses and restored mitochondrial function in a headache mouse model via Sirt3/Pgc-1α positive feedback loop.
Not available
S-EPMC10240765 | biostudies-literature
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