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Although the severe systemic consequences of the disorder are becoming clear, the molecular effects are not well defined. Therefore, for further elucidating the effects of ETHE1\u2010deficiency, we performed a large scale quantitative proteomics study on liver tissue from ETHE1\u2010deficient mice. Our results demonstrated a clear link between ETHE1\u2010deficiency and redox active proteins, as reflected by downregulation of several proteins related to oxidation\u2010reduction, such as different dehydrogenases and cytochrome P450 (CYP450) members. Furthermore, the protein data indicated impact of the ETHE1\u2010deficiency on metabolic reprogramming through upregulation of glycolytic enzymes and by altering several heterogeneous ribonucleoproteins, indicating novel link between ETHE1 and gene expression regulation. We also found increase in total protein acetylation level, pointing out the link between ETHE1 and acetylation, which is likely controlled by both redox state and cellular metabolites. These findings are relevant for understanding the complexity of the disease and may shed light on important functions influenced by ETHE1 deficiency and by the concomitant increase in the gaseous mediator hydrogen sulfide. All MS data have been deposited in the ProteomeXchange with the dataset identifiers PXD002741 (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/proteomecentral.proteomexchange.org\/dataset\/PXD002741\">http:\/\/proteomecentral.proteomexchange.org\/dataset\/PXD002741<\/jats:ext-link>) and PXD002742 (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/proteomecentral.proteomexchange.org\/dataset\/PXD002741\">http:\/\/proteomecentral.proteomexchange.org\/dataset\/PXD002741<\/jats:ext-link>).<\/jats:p>","DOI":"10.1002\/pmic.201500336","type":"journal-article","created":{"date-parts":[[2016,2,12]],"date-time":"2016-02-12T03:12:54Z","timestamp":1455246774000},"page":"1166-1176","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Quantitative proteomics suggests metabolic reprogramming during ETHE1 deficiency"],"prefix":"10.1002","volume":"16","author":[{"given":"Navid","family":"Sahebekhtiari","sequence":"first","affiliation":[{"name":"Research Unit for Molecular Medicine, Department of Clinical Medicine Aarhus University and Aarhus University Hospital  Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle M.","family":"Thomsen","sequence":"additional","affiliation":[{"name":"Research Unit for Molecular Medicine, Department of Clinical Medicine Aarhus University and Aarhus University Hospital  Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens J.","family":"Sloth","sequence":"additional","affiliation":[{"name":"Research Group for NanoBio Science, National Food Institute Technical University of Denmark  S\u00f8borg Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vibeke","family":"Stenbroen","sequence":"additional","affiliation":[{"name":"Research Unit for Molecular Medicine, Department of Clinical Medicine Aarhus University and Aarhus University Hospital  Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Massimo","family":"Zeviani","sequence":"additional","affiliation":[{"name":"Mitochondrial Biology Unit Medical Research Council Wellcome Trust\/MRC Building  Cambridge UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niels","family":"Gregersen","sequence":"additional","affiliation":[{"name":"Research Unit for Molecular Medicine, Department of Clinical Medicine Aarhus University and Aarhus University Hospital  Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlo","family":"Viscomi","sequence":"additional","affiliation":[{"name":"Mitochondrial Biology Unit Medical Research Council Wellcome Trust\/MRC Building  Cambridge UK"},{"name":"IRCCS Foundation Neurological Institute \u201cC. 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