2006
DOI: 10.1016/j.jmb.2006.06.057
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Mitochondrial Respiratory Chain Supercomplexes Are Destabilized in Barth Syndrome Patients

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Cited by 381 publications
(318 citation statements)
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“…Given the importance of CL in the proper functioning of many of the components of the oxidative phosphorylation machinery, a reasonable hypothesis for the defects in respiration in BTHS patients, and models alike, is that reduced CL content, altered acyl chain content, and/or the increased abundance of MLCL destabilize respiratory supercomplexes resulting in reduced respiratory efficiency. Consistent with this postulate, the assembly and stability of respiratory supercomplexes is compromised in fibroblasts derived from BTHS patients (McKenzie et al, 2006) and in one yeast BTHS model (Brandner et al, 2005). However, we recently demonstrated in another yeast BTHS model (a different strain than used in Brandner et al, 2005), that although there are moderate but significant changes in respiratory supercomplex function, there are no discernable defects in respiratory supercomplex assembly in the absence of Taz1p (Claypool et al, 2008).…”
Section: Introductionsupporting
confidence: 54%
“…Given the importance of CL in the proper functioning of many of the components of the oxidative phosphorylation machinery, a reasonable hypothesis for the defects in respiration in BTHS patients, and models alike, is that reduced CL content, altered acyl chain content, and/or the increased abundance of MLCL destabilize respiratory supercomplexes resulting in reduced respiratory efficiency. Consistent with this postulate, the assembly and stability of respiratory supercomplexes is compromised in fibroblasts derived from BTHS patients (McKenzie et al, 2006) and in one yeast BTHS model (Brandner et al, 2005). However, we recently demonstrated in another yeast BTHS model (a different strain than used in Brandner et al, 2005), that although there are moderate but significant changes in respiratory supercomplex function, there are no discernable defects in respiratory supercomplex assembly in the absence of Taz1p (Claypool et al, 2008).…”
Section: Introductionsupporting
confidence: 54%
“…It has also been shown that Tafazzin defects result in destabilized OXPHOS supercomplexes , which in turn results in reduced steady-state levels of CI. These findings suggest that cardiolipin is essential for OXPHOS supercomplex stability, and that loss of supercomplex stability contributes to Barth syndrome pathogenesis [19].…”
Section: Oxphos Supercomplexesmentioning
confidence: 82%
“…In addition, the phospholipid cardiolipin is required for OX-PHOS supercomplex assembly and stability [19]. The majority of cardiolipin is found in the inner mitochondrial membrane where it is essential for mitochondrial function.…”
Section: Oxphos Supercomplexesmentioning
confidence: 99%
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