Sort   by:  
 Page size 
ProteomicsLoss of COX4i1 leads to combined respiratory chain deficiency and impaired mitochondrial protein synthesis
The oxidative phosphorylation (OXPHOS) system localized in the inner mitochondrial membrane secures production of the majority of ATP in mammalian organisms. Individual OXPHOS complexes form supramolecular assemblies termed supercomplexes. The complexes are linked not only by their function but also...
ORGANISM(S): Homo sapiens (Human) 
2021-02-18 | PXD023367 | Pride
Knock-out Cox Mitochondria Oxphos Complex i Cox assembly Mitochondrial protein synthesis Cox4 Biogenesis interdependency Complexome profiling 
ProteomicsEbselen as a promising treatment in mitochondrial diseases: focus on Barth syndrome and complex I deficiencies
Over the past decade, significant progress improved mitochondrial disorders diagnosis, but effective therapies remain lacking. A drug screening identified Ebselen (EBS) as enhancing oxidative phosphorylation-dependent growth in a yeast model of Barth syndrome, a cardiomyopathy caused by defective ca...
ORGANISM(S): Mus musculus (Mouse) Homo sapiens (Human) Saccharomyces cerevisiae (Baker's yeast) 
2026-09-03 | PXD072219 | Pride
Barth syndrome  mitochondrial diseases  drug repositioning  ebselen  krebs cycle  cytosolic translation 
UnknownLoss of COX4I1 Leads to Combined Respiratory Chain Deficiency and Impaired Mitochondrial Protein Synthesis.
Not available
S-EPMC7916595 | biostudies-literature
UnknownWhole-exome sequencing identifies novel variants in PNPT1 causing oxidative phosphorylation defects and severe multisystem disease.
Not available
S-EPMC5159763 | biostudies-literature
ProteomicsMitochondrial Network Organization during Neuronal Reprogramming, MT_UDF_SEC
The study focuses on an extensive biochemical fractionation with in-depth quantitative mass spectrometric profiling in the mitochondrial (mt) extracts of cultured human NTera2 embryonal carcinoma stem cells (i.e. ECSCs or undifferentiated state) and upon exposure to retinoic acid-induced differentia...
ORGANISM(S): Homo sapiens (Human) 
2020-01-09 | PXD009834 | Pride
Biochemical fractionation  ci and civ deficiencies  differentiated neuronal cells  human ntera2 embryonal carcinoma stem cells  mass spectrometry  mitochondrial proteins  mitochondrial interaction landscape  proteomics  protein complexes  patient fibroblasts  neurodegeneration  orphans 
ProteomicsMitochondrial Network Organization during Neuronal Reprogramming, MT-DF-WAX
The study focuses on an extensive biochemical fractionation with in-depth quantitative mass spectrometric profiling in the mitochondrial (mt) extracts of cultured human NTera2 embryonal carcinoma stem cells (i.e. ECSCs or undifferentiated state) and upon exposure to retinoic acid-induced differentia...
ORGANISM(S): Homo sapiens (Human) 
2020-01-09 | PXD009831 | Pride
Biochemical fractionation  ci and civ deficiencies  differentiated neuronal cells  human ntera2 embryonal carcinoma stem cells  mass spectrometry  mitochondrial proteins  mitochondrial interaction landscape  proteomics  protein complexes  patient fibroblasts  neurodegeneration  orphans 
ProteomicsMitochondrial Network Organization during Neuronal Reprogramming, MT_DF_SEC
The study focuses on an extensive biochemical fractionation with in-depth quantitative mass spectrometric profiling in the mitochondrial (mt) extracts of cultured human NTera2 embryonal carcinoma stem cells (i.e. ECSCs or undifferentiated state) and upon exposure to retinoic acid-induced differentia...
ORGANISM(S): Homo sapiens (Human) 
2020-01-09 | PXD009833 | Pride
Biochemical fractionation  ci and civ deficiencies  differentiated neuronal cells  human ntera2 embryonal carcinoma stem cells  mass spectrometry  mitochondrial proteins  mitochondrial interaction landscape  proteomics  protein complexes  patient fibroblasts  neurodegeneration  orphans 
ProteomicsMitochondrial Network Organization during Neuronal Reprogramming, MT_UDF_WAX
The study focuses on an extensive biochemical fractionation with in-depth quantitative mass spectrometric profiling in the mitochondrial (mt) extracts of cultured human NTera2 embryonal carcinoma stem cells (i.e. ECSCs or undifferentiated state) and upon exposure to retinoic acid-induced differentia...
ORGANISM(S): Homo sapiens (Human) 
2020-01-09 | PXD009832 | Pride
Biochemical fractionation  ci and civ deficiencies  differentiated neuronal cells  human ntera2 embryonal carcinoma stem cells  mass spectrometry  mitochondrial proteins  mitochondrial interaction landscape  proteomics  protein complexes  patient fibroblasts  neurodegeneration  orphans 
TranscriptomicsGene expression profiling in equine polysaccharide storage myopathy
Standardized muscular biopsies of the dorsal compartment of the gluteus medius muscle were performed in 7 horses suffering from equine polysaccharide storage myopathy (PSSM) and 6 sound Norman Cob horses . Gene expression analysis was performed using an equine oligonucleotide microarray which includ...
ORGANISM(S): Equus caballus 
2009-04-08 | E-GEOD-15572 | biostudies-arrayexpress
Sort   by:  
 Page size