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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Boutant, Marie Taleux, Nellie Arnauné-pelloquin, Laeticia Gottlieb, Eyal Vial, Guillaume Manfredi, Giovanni Landes, Thomas Slomianny, Christian Puech, Jean-philippe Moustapha, Aoula Houtkooper, Riekelt H. Bellenguer, Pascale Vaz, Frederic M. Petit, Patrice X. Wanders, Ronald J. Møller, Ian Max Gonzalvez, François D'aurelio, Marilena |
| Description | Author Affiliation: Gonzalvez F ( INSERM U-747 et Université Paris V-Descartes, Centre de Recherche des Saint-Pères, 45 Rue des Saint-Pères, 75006 Paris, France.) |
| Abstract | Cardiolipin is a mitochondrion-specific phospholipid that stabilizes the assembly of respiratory chain complexes, favoring full-yield operation. It also mediates key steps in apoptosis. In Barth syndrome, an X chromosome-linked cardiomyopathy caused by tafazzin mutations, cardiolipins display acyl chain modifications and are present at abnormally low concentrations, whereas monolysocardiolipin accumulates. Using immortalized lymphoblasts from Barth syndrome patients, we showed that the production of abnormal cardiolipin led to mitochondrial alterations. Indeed, the lack of normal cardiolipin led to changes in electron transport chain stability, resulting in cellular defects. We found a destabilization of the supercomplex (respirasome) I + III $_{2}$ + IV $_{n}$ but also decreased amounts of individual complexes I and IV and supercomplexes I + III and III + IV. No changes were observed in the amounts of individual complex III and complex II. We also found decreased levels of complex V. This complex is not part of the supercomplex suggesting that cardiolipin is required not only for the association/stabilization of the complexes into supercomplexes but also for the modulation of the amount of individual respiratory chain complexes. However, these alterations were compensated by an increase in mitochondrial mass, as demonstrated by electron microscopy and measurements of citrate synthase activity. We suggest that this compensatory increase in mitochondrial content prevents a decrease in mitochondrial respiration and ATP synthesis in the cells. We also show, by extensive flow cytometry analysis, that the type II apoptosis pathway was blocked at the mitochondrial level and that the mitochondria of patients with Barth syndrome cannot bind active caspase-8. Signal transduction is thus blocked before any mitochondrial event can occur. Remarkably, basal levels of superoxide anion production were slightly higher in patients' cells than in control cells as previously evidenced via an increased protein carbonylation in the taz1 Δ mutant in the yeast. This may be deleterious to cells in the long term. The consequences of mitochondrial dysfunction and alterations to apoptosis signal transduction are considered in light of the potential for the development of future treatments. |
| ISSN | 00063002 |
| Journal | Biochimica et Biophysica Acta (BBA) - Reviews on Cancer |
| Issue Number | 8 |
| Volume Number | 1832 |
| Language | English |
| Publisher | Elsevier |
| Publisher Date | 2013-08-01 |
| Publisher Place | Netherlands |
| Access Restriction | Open |
| Subject Keyword | Apoptosis Genetics Barth Syndrome Pathology Cardiolipins Metabolism Mitochondria Mutation Transcription Factors Adenosine Triphosphate Caspase 8 Cell Death Cell Line Citrate (si)-Synthase Electron Transport Chain Complex Proteins Lymphocytes Lysophospholipids Signal Transduction Superoxides Biochemistry |
| Content Type | Text |
| Resource Type | Article |
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