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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
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
MetabolomicsATAD3A has a scaffolding role regulating mitochondria inner membrane structure and protein assembly

The ATPase Family AAA Domain Containing 3A (ATAD3A), is a mitochondrial inner membrane protein conserved in metazoans. ATAD3A has been associated with several mitochondrial functions, including nucleoid organization, cholesterol metabolism, and mitochondrial translation. To address its primary ro...

2022-01-14 | MTBLS3786 | MetaboLights
ProteomicsRTN4IP1 is essential for the final stages of mitochondrial complex I assembly
A biochemical deficiency of mitochondrial complex I (CI) underlies ~30% of cases of primary mitochondrial disease, yet the inventory of molecular machinery required for CI assembly remains incomplete. We previously characterised patients with isolated CI deficiency caused by segregating variants in ...
ORGANISM(S): Homo sapiens (Human) 
2025-08-08 | PXD055511 | Pride
Oxidative phosphorylation system Lc-msms Complexome profiling Complex i assembly 
ProteomicsDissecting the Roles of Mitochondrial Complex I Intermediate Assembly (MCIA) Complex Factors in the Biogenesis of Complex I
Mitochondrial Complex I harbors 7 mitochondrial and 38 nuclear-encoded subunits. Its biogenesis requires the assembly and integration of distinct intermediate modules, mediated by numerous assembly factors. The Mitochondrial Complex I Intermediate Assembly (MCIA) complex, containing assembly factors...
ORGANISM(S): Homo sapiens (Human) 
2020-04-22 | PXD018075 | Pride
Assembly factors Mitochondria Nadh-ubiquinone dehydrogenase Mcia complex Complex i Oxidative phosphorylation 
ProteomicsOptic Atrophy-associated TMEM126A is an assembly factor for the ND4-module of Mitochondrial Complex I
Mitochondrial disease is a debilitating condition with a diverse genetic aetiology. Here, we report that TMEM126A, a protein that is mutated in patients with autosomal recessive optic atrophy, participates directly in the assembly of mitochondrial complex I. Using a combination of genome editing, in...
ORGANISM(S): Homo sapiens (Human) 
2021-04-21 | PXD023136 | Pride
Assembly factor Complex i Tmem126a Mitochondria 
GenomicsH3K4me3 Interactions with TAF3 Regulate Preinitiation Complex Assembly and Selective Gene Activation [ChIP-Seq]
Histone modifications regulate chromatin-dependent processes, yet the mechanisms by which they contribute to specific outcomes remain unclear. H3K4me3 is a prominent histone mark that is associated with active genes and promotes transcription through interactions with effector proteins that include ...
ORGANISM(S): Homo sapiens 
2013-03-02 | E-GEOD-43539 | biostudies-arrayexpress
ProteomicsThe assembly of the Mitochondrial Complex I Assembly complex uncovers a redox pathway coordination
The Mitochondrial Complex I Assembly (MCIA) complex is essential for the biogenesis of respiratory Complex I (CI), the first enzyme in the respiratory chain, which has been linked to Alzheimer’s disease (AD) pathogenesis. However, how MCIA facilitates CI assembly, and how it is linked with AD pathog...
ORGANISM(S): Homo sapiens (Human) Escherichia coli 
2023-12-13 | PXD042905 | Pride
Keywords: oxidative phosphorylation (oxphos) Mitochondrial complex i assembly complex (mcia) Fatty acid oxidation (fao) Amyloid-β Ecsit phosphorylation Cryo-em 
GenomicsEic1 links Mis18 with the CCAN/Mis6/Ctf19 complex to promote CENP-A assembly
CENP-A chromatin the foundation for kinetochore assembly in all eukaryotes. CENP-A is incorporated outside of S phase and depends on its chaperone HJURPScm3, and Mis18 in vertebrates and fission yeast. The recruitment of Mis18 and HJURPScm3 to centromeres is cell cycle regulated. Vertebrate Mis18 a...
ORGANISM(S): Schizosaccharomyces pombe 
2014-05-19 | E-GEOD-54685 | biostudies-arrayexpress
TranscriptomicsH3K4me3 Interactions with TAF3 Regulate Preinitiation Complex Assembly and Selective Gene Activation [HIT-Seq]
Histone modifications regulate chromatin-dependent processes, yet the mechanisms by which they contribute to specific outcomes remain unclear. H3K4me3 is a prominent histone mark that is associated with active genes and promotes transcription through interactions with effector proteins that include ...
ORGANISM(S): Mus musculus 
2013-03-02 | E-GEOD-43540 | biostudies-arrayexpress
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