Barth syndrome: cellular compensation of mitochondrial dysfunction and apoptosis inhibition due to changes in cardiolipin remodeling linked to tafazzin (TAZ) gene mutation.

@article{Gonzalvez2013BarthSC,
  title={Barth syndrome: cellular compensation of mitochondrial dysfunction and apoptosis inhibition due to changes in cardiolipin remodeling linked to tafazzin (TAZ) gene mutation.},
  author={François Gonzalvez and Marilena D'Aurelio and Marie Boutant and Aoula Moustapha and Jean-Philippe Puech and Thomas Landes and Laeticia Arnaun{\'e}-Pelloquin and Guillaume Vial and Nellie Taleux and Christian Slomianny and Ronald Wanders and Riekelt H Houtkooper and Pascale Bellenguer and Ian Max M\oller and Eyal Gottlieb and Fr{\'e}d{\'e}ric M Vaz and Giovanni Manfredi and Patrice Xavier Petit},
  journal={Biochimica et biophysica acta},
  year={2013},
  volume={1832 8},
  pages={1194-206}
}
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… CONTINUE READING
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