Grunau, Silke D.

[["dc.bibliographiccitation.firstpage","139"],["dc.bibliographiccitation.issue","2"],["dc.bibliographiccitation.journal","EMBO Molecular Medicine"],["dc.bibliographiccitation.lastpage","154"],["dc.bibliographiccitation.volume","8"],["dc.contributor.author","Dudek, Jan"],["dc.contributor.author","Cheng, I-Fen"],["dc.contributor.author","Chowdhury, Arpita"],["dc.contributor.author","Wozny, Katharina"],["dc.contributor.author","Balleininger, Martina"],["dc.contributor.author","Reinhold, Robert"],["dc.contributor.author","Grunau, Silke"],["dc.contributor.author","Callegari, Sylvie"],["dc.contributor.author","Toischer, Karl"],["dc.contributor.author","Wanders, Ronald JA"],["dc.contributor.author","Hasenfuß, Gerd"],["dc.contributor.author","Brügger, Britta"],["dc.contributor.author","Guan, Kaomei"],["dc.contributor.author","Rehling, Peter"],["dc.date.accessioned","2017-09-07T11:53:31Z"],["dc.date.available","2017-09-07T11:53:31Z"],["dc.date.issued","2016"],["dc.description.abstract","Barth syndrome (BTHS) is a cardiomyopathy caused by the loss of tafazzin, a mitochondrial acyltransferase involved in the maturation of the glycerophospholipid cardiolipin. It has remained enigmatic as to why a systemic loss of cardiolipin leads to cardiomyopathy. Using a genetic ablation of tafazzin function in the BTHS mouse model, we identified severe structural changes in respiratory chain supercomplexes at a pre‐onset stage of the disease. This reorganization of supercomplexes was specific to cardiac tissue and could be recapitulated in cardiomyocytes derived from BTHS patients. Moreover, our analyses demonstrate a cardiac‐specific loss of succinate dehydrogenase (SDH), an enzyme linking the respiratory chain with the tricarboxylic acid cycle. As a similar defect of SDH is apparent in patient cell‐derived cardiomyocytes, we conclude that these defects represent a molecular basis for the cardiac pathology in Barth syndrome."],["dc.identifier.doi","10.15252/emmm.201505644"],["dc.identifier.fs","615879"],["dc.identifier.gro","3145083"],["dc.identifier.pmid","26697888"],["dc.identifier.purl","https://resolver.sub.uni-goettingen.de/purl?gs-1/13136"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/2780"],["dc.identifier.url","https://sfb1002.med.uni-goettingen.de/production/literature/publications/101"],["dc.language.iso","en"],["dc.notes.intern","Crossref Import"],["dc.notes.intern","Merged from goescholar"],["dc.notes.status","final"],["dc.relation","SFB 1002: Modulatorische Einheiten bei Herzinsuffizienz"],["dc.relation","SFB 1002 | A06: Molekulare Grundlagen mitochondrialer Kardiomyopathien"],["dc.relation.issn","1757-4676"],["dc.relation.issn","1757-4684"],["dc.relation.workinggroup","RG Guan (Application of patient-specific induced pluripotent stem cells in disease modelling)"],["dc.relation.workinggroup","RG Hasenfuß (Transition zur Herzinsuffizienz)"],["dc.relation.workinggroup","RG Rehling (Mitochondrial Protein Biogenesis)"],["dc.relation.workinggroup","RG Toischer (Kardiales Remodeling)"],["dc.rights","CC BY 4.0"],["dc.rights.uri","https://creativecommons.org/licenses/by/4.0"],["dc.subject","Barth syndrome; Cardiolipin, Mitochondriar; Respiratory chain; Succinate dehydrogenase"],["dc.title","Cardiac-specific succinate dehydrogenase deficiency in Barth syndrome"],["dc.type","journal_article"],["dc.type.internalPublication","unknown"],["dc.type.peerReviewed","no"],["dc.type.subtype","original_ja"],["dc.type.version","published_version"],["dspace.entity.type","Publication"]]