Vukotic, Milena

[["dc.bibliographiccitation.firstpage","806"],["dc.bibliographiccitation.issue","2"],["dc.bibliographiccitation.journal","Stem Cell Research"],["dc.bibliographiccitation.lastpage","819"],["dc.bibliographiccitation.volume","11"],["dc.contributor.author","Dudek, Jan"],["dc.contributor.author","Cheng, I-Fen"],["dc.contributor.author","Balleininger, Martina"],["dc.contributor.author","Vaz, Frederic M."],["dc.contributor.author","Streckfuss-Bömeke, Katrin"],["dc.contributor.author","Hübscher, Daniela"],["dc.contributor.author","Vukotic, Milena"],["dc.contributor.author","Wanders, Ronald J. A."],["dc.contributor.author","Rehling, Peter"],["dc.contributor.author","Guan, Kaomei"],["dc.date.accessioned","2017-09-07T11:47:37Z"],["dc.date.available","2017-09-07T11:47:37Z"],["dc.date.issued","2013"],["dc.description.abstract","Barth syndrome (BTHS) patients carrying mutations in tafazzin (TAZ1), which is involved in the final maturation of cardiolipin, present with dilated cardiomyopathy, skeletal myopathy, growth retardation and neutropenia. To study how mitochondrial function is impaired in BTHS patients, we generated induced pluripotent stem cells (iPSCs) to develop a novel and relevant human model system for BTHS. BTHS-iPSCs generated from dermal fibroblasts of three patients with different mutations in TAZ1 expressed pluripotency markers, and were able to differentiate into cells derived from all three germ layers both in vitro and in vivo. We used these cells to study the impact of tafazzin deficiency on mitochondria( oxidative phosphorylation. We found an impaired remodeling of cardiolipin, a dramatic decrease in basal oxygen consumption rate and in the maximal respiratory capacity in BTHS-iPSCs. Simultaneous measurement of extra-cellular acidification rate allowed us a thorough assessment of the metabolic deficiency in BTHS patients. Blue native gel analyses revealed that decreased respiration coincided with dramatic structural changes in respiratory chain supercomplexes leading to a massive increase in generation of reactive oxygen species. Our data demonstrate that BTHS-iPSCs are capable of modeling BTHS by recapitulating the disease phenotype and thus are important tools for studying the disease mechanism. (C) 2013 The Authors. Published by Elsevier B.V. All rights reserved."],["dc.identifier.doi","10.1016/j.scr.2013.05.005"],["dc.identifier.gro","3142297"],["dc.identifier.isi","000323586600012"],["dc.identifier.pmid","23792436"],["dc.identifier.purl","https://resolver.sub.uni-goettingen.de/purl?gs-1/11333"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/6720"],["dc.identifier.url","https://sfb1002.med.uni-goettingen.de/production/literature/publications/12"],["dc.language.iso","en"],["dc.notes.intern","WoS Import 2017-03-10"],["dc.notes.intern","Merged from goescholar"],["dc.notes.status","final"],["dc.notes.submitter","PUB_WoS_Import"],["dc.relation","SFB 1002: Modulatorische Einheiten bei Herzinsuffizienz"],["dc.relation","SFB 1002 | A04: Patienten-spezifische induzierte pluripotente Stammzellen zur funktionellen Untersuchung von Ryanodinrezeptor-Mutationen"],["dc.relation","SFB 1002 | A06: Molekulare Grundlagen mitochondrialer Kardiomyopathien"],["dc.relation.issn","1873-5061"],["dc.relation.workinggroup","RG Guan (Application of patient-specific induced pluripotent stem cells in disease modelling)"],["dc.relation.workinggroup","RG Rehling (Mitochondrial Protein Biogenesis)"],["dc.rights","Goescholar"],["dc.rights.uri","https://goescholar.uni-goettingen.de/licenses"],["dc.title","Cardiolipin deficiency affects respiratory chain function and organization in an induced pluripotent stem cell model of Barth syndrome"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original_ja"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","2985"],["dc.bibliographiccitation.issue","17"],["dc.bibliographiccitation.journal","FEBS Letters"],["dc.bibliographiccitation.lastpage","2992"],["dc.bibliographiccitation.volume","588"],["dc.contributor.author","Deckers, Markus"],["dc.contributor.author","Balleininger, Martina"],["dc.contributor.author","Vukotic, Milena"],["dc.contributor.author","Römpler, Katharina"],["dc.contributor.author","Bareth, Bettina"],["dc.contributor.author","Juris, Lisa"],["dc.contributor.author","Dudek, Jan"],["dc.date.accessioned","2018-11-07T09:36:26Z"],["dc.date.available","2018-11-07T09:36:26Z"],["dc.date.issued","2014"],["dc.description.abstract","The mitochondrial respiratory chain is essential for the conversion of energy derived from the oxidation of metabolites into the membrane potential, which drives the synthesis of ATP. The electron transporting complexes bc(1) complex and the cytochrome c oxidase assemble into large supercomplexes, allowing efficient energy transduction. Currently, we have only limited information about what determines the structure of the supercomplex. Here, we characterize Aim24 in baker's yeast as a protein, which is integrated in the mitochondrial inner membrane and is required for the structural integrity of the supercomplex. Deletion of AIM24 strongly affects activity of the respiratory chain and induces a growth defect on non-fermentable medium. Our data indicate that Aim24 has a function in stabilizing the respiratory chain supercomplexes. (C) 2014 Federation of European Biochemical Societies. Published by Elsevier B. V. All rights reserved."],["dc.description.sponsorship","Deutsche Forschungsgemeinschaft [SFB 1002]"],["dc.identifier.doi","10.1016/j.febslet.2014.06.006"],["dc.identifier.isi","000340882900032"],["dc.identifier.pmid","24928273"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/32621"],["dc.identifier.url","https://sfb1002.med.uni-goettingen.de/production/literature/publications/8"],["dc.language.iso","en"],["dc.notes.status","final"],["dc.notes.submitter","Najko"],["dc.relation","SFB 1002: Modulatorische Einheiten bei Herzinsuffizienz"],["dc.relation","SFB 1002 | A06: Molekulare Grundlagen mitochondrialer Kardiomyopathien"],["dc.relation.issn","1873-3468"],["dc.relation.issn","0014-5793"],["dc.relation.workinggroup","RG Rehling (Mitochondrial Protein Biogenesis)"],["dc.title","Aim24 stabilizes respiratory chain supercomplexes and is required for efficient respiration"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original_ja"],["dspace.entity.type","Publication"]]