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ProteomicsQuantitative Proteomics Reveals Metabolic Reprogramming during ETHE1 Deficiency, Part 2
Deficiency of mitochondrial sulfur dioxygenase (ETHE1) causes the severe metabolic disorder ethylmalonic encephalopathy, which is characterized by early-onset encephalopathy and defective cytochrome C oxidase because of hydrogen sulfide accumulation. Although the severe systemic consequences of the ...
ORGANISM(S): Mus musculus (Mouse) 
2016-04-11 | PXD002742 | Pride
Metabolism Persulfide dioxygenase Acetylation Sulfide Liver Mouse Ethe1 Itraq Lc-ms/ms 
ProteomicsQuantitative Proteomics Reveals Metabolic Reprogramming during ETHE1 Deficiency, Part 1
Deficiency of mitochondrial sulfur dioxygenase (ETHE1) causes the severe metabolic disorder ethylmalonic encephalopathy, which is characterized by early-onset encephalopathy and defective cytochrome C oxidase because of hydrogen sulfide accumulation. Although the severe systemic consequences of the ...
ORGANISM(S): Mus musculus (Mouse) 
2016-04-11 | PXD002741 | Pride
Metabolism Persulfide dioxygenase Acetylation Sulfide Liver Mouse Ethe1 Itraq Lc-ms/ms 
ProteomicsLC-MS/MS proteomics of ETHE1 deficient patient fibroblasts_Study-1
The mitochondrial enzyme ETHE1 is a persulfide dioxygenase essential for cellular sulfide detoxification, and its deficiency causes the severe and complex inherited metabolic disorder ethylmalonic encephalopathy (EE). In spite of well-described clinical symptoms of the disease, detailed cellular and...
ORGANISM(S): Homo sapiens (Human) 
2018-11-07 | PXD009880 | Pride
Patient fibroblasts Ethe1 deficiency Tmt labeling Lc-ms/ms ethylmalonic acid encephalopathy 
UnknownETHE1 and MOCS1 deficiencies: Disruption of mitochondrial bioenergetics, dynamics, redox homeostasis and endoplasmic reticulum-mitochondria crosstalk in patient fibroblasts.
Not available
S-EPMC6718683 | biostudies-literature
UnknownThe Role of Persulfide Metabolism During Arabidopsis Seed Development Under Light and Dark Conditions.
Not available
S-EPMC6156424 | biostudies-literature
UnknownMitochondrial Unfolded Protein Response (mtUPR) Activation Improves Pathological Alterations in Cellular Models of Ethylmalonic Encephalopathy.
Not available
S-EPMC12189359 | biostudies-literature
UnknownImproved clinical outcome following liver transplant in patients with ethylmalonic encephalopathy.
Not available
S-EPMC9873405 | biostudies-literature
TranscriptomicsPeripheral blood mononuclear cells gene expression reflects adaptive response and metabolic damage associated to the intake of diets with an unbalanced proportion of macronutrients
Gene expression studies in peripheral blood mononuclear cells (PBMC) can provide knowledge that would be difficult to obtain using other types of biological samples, as these cells can reflect overall response of the body to a specific stimulus, such as diet. Here, we aimed to study the impact of su...
ORGANISM(S): Rattus norvegicus 
2015-12-07 | E-GEOD-54897 | biostudies-arrayexpress
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