Deckers, Markus

[["dc.bibliographiccitation.artnumber","e9561"],["dc.bibliographiccitation.issue","5"],["dc.bibliographiccitation.journal","EMBO Molecular Medicine"],["dc.bibliographiccitation.volume","11"],["dc.contributor.author","Mohanraj, Karthik"],["dc.contributor.author","Wasilewski, Michal"],["dc.contributor.author","Benincá, Cristiane"],["dc.contributor.author","Cysewski, Dominik"],["dc.contributor.author","Poznanski, Jaroslaw"],["dc.contributor.author","Sakowska, Paulina"],["dc.contributor.author","Bugajska, Zaneta"],["dc.contributor.author","Deckers, Markus"],["dc.contributor.author","Dennerlein, Sven"],["dc.contributor.author","Fernandez‐Vizarra, Erika"],["dc.contributor.author","Rehling, Peter"],["dc.contributor.author","Dadlez, Michal"],["dc.contributor.author","Zeviani, Massimo"],["dc.contributor.author","Chacinska, Agnieszka"],["dc.date.accessioned","2019-07-09T11:51:37Z"],["dc.date.available","2019-07-09T11:51:37Z"],["dc.date.issued","2019"],["dc.description.abstract","Nuclear and mitochondrial genome mutations lead to various mitochondrial diseases, many of which affect the mitochondrial respiratory chain. The proteome of the intermembrane space (IMS) of mitochondria consists of several important assembly factors that participate in the biogenesis of mitochondrial respiratory chain complexes. The present study comprehensively analyzed a recently identified IMS protein cytochrome c oxidase assembly factor 7 (COA7), or RESpiratory chain Assembly 1 (RESA1) factor that is associated with a rare form of mitochondrial leukoencephalopathy and complex IV deficiency. We found that COA7 requires the mitochondrial IMS import and assembly (MIA) pathway for efficient accumulation in the IMS. We also found that pathogenic mutant versions of COA7 are imported slower than the wild‐type protein, and mislocalized proteins are degraded in the cytosol by the proteasome. Interestingly, proteasome inhibition rescued both the mitochondrial localization of COA7 and complex IV activity in patient‐derived fibroblasts. We propose proteasome inhibition as a novel therapeutic approach for a broad range of mitochondrial pathologies associated with the decreased levels of mitochondrial proteins."],["dc.identifier.doi","10.15252/emmm.201809561"],["dc.identifier.pmid","30885959"],["dc.identifier.purl","https://resolver.sub.uni-goettingen.de/purl?gs-1/16155"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/59974"],["dc.identifier.url","https://sfb1190.med.uni-goettingen.de/production/literature/publications/64"],["dc.language.iso","en"],["dc.notes.intern","Merged from goescholar"],["dc.relation","info:eu-repo/grantAgreement/EC/FP7/322424/EU//MITCARE"],["dc.relation","info:eu-repo/grantAgreement/EC/FP7/339580/EU//MITRAC"],["dc.relation","SFB 1190: Transportmaschinen und Kontaktstellen zellulärer Kompartimente"],["dc.relation","SFB 1190 | P13: Protein Transport über den mitochondrialen Carrier Transportweg"],["dc.relation.workinggroup","RG Rehling (Mitochondrial Protein Biogenesis)"],["dc.rights","CC BY 4.0"],["dc.rights.uri","https://creativecommons.org/licenses/by/4.0"],["dc.subject.ddc","540"],["dc.title","Inhibition of proteasome rescues a pathogenic variant of respiratory chain assembly factor COA7"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.subtype","original_ja"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","1083"],["dc.bibliographiccitation.issue","7"],["dc.bibliographiccitation.journal","The Journal of Cell Biology"],["dc.bibliographiccitation.lastpage","1086"],["dc.bibliographiccitation.volume","204"],["dc.contributor.author","Pfanner, Nikolaus"],["dc.contributor.author","van der Laan, Martin"],["dc.contributor.author","Amati, Paolo"],["dc.contributor.author","Capaldi, Roderick A."],["dc.contributor.author","Caudy, Amy A."],["dc.contributor.author","Chacinska, Agnieszka"],["dc.contributor.author","Darshi, Manjula"],["dc.contributor.author","Deckers, Markus"],["dc.contributor.author","Hoppins, Suzanne"],["dc.contributor.author","Icho, Tateo"],["dc.contributor.author","Jakobs, Stefan"],["dc.contributor.author","Ji, Jianguo"],["dc.contributor.author","Kozjak-Pavlovic, Vera"],["dc.contributor.author","Meisinger, Chris"],["dc.contributor.author","Odgren, Paul R."],["dc.contributor.author","Park, Sang Ki"],["dc.contributor.author","Rehling, Peter"],["dc.contributor.author","Reichert, Andreas S."],["dc.contributor.author","Sheikh, M. Saeed"],["dc.contributor.author","Taylor, Susan S."],["dc.contributor.author","Tsuchida, Nobuo"],["dc.contributor.author","van der Bliek, Alexander M."],["dc.contributor.author","van der Klei, Ida J."],["dc.contributor.author","Weissman, Jonathan S."],["dc.contributor.author","Westermann, Benedikt"],["dc.contributor.author","Zha, Jiping"],["dc.contributor.author","Neupert, Walter"],["dc.contributor.author","Nunnari, Jodi"],["dc.date.accessioned","2017-09-07T11:46:22Z"],["dc.date.available","2017-09-07T11:46:22Z"],["dc.date.issued","2014"],["dc.description.abstract","The mitochondrial inner membrane contains a large protein complex that functions in inner membrane organization and formation of membrane contact sites. The complex was variably named the mitochondrial contact site complex, mitochondrial inner membrane organizing system, mitochondrial organizing structure, or Mitofilin/Fcj1 complex. To facilitate future studies, we propose to unify the nomenclature and term the complex \"mitochondrial contact site and cristae organizing system\" and its subunits Mic10 to Mic60."],["dc.identifier.doi","10.1083/jcb.201401006"],["dc.identifier.gro","3142162"],["dc.identifier.isi","000333903600003"],["dc.identifier.pmid","24687277"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/5221"],["dc.language.iso","en"],["dc.notes.intern","WoS Import 2017-03-10 / Funder: NIGMS NIH HHS [R01 GM062942, R01 GM097432]"],["dc.notes.status","final"],["dc.notes.submitter","PUB_WoS_Import"],["dc.relation.eissn","1540-8140"],["dc.relation.issn","0021-9525"],["dc.title","Uniform nomenclature for the mitochondrial contact site and cristae organizing system"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dspace.entity.type","Publication"]]