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

doi:10.1016/j.bbadis.2013.03.005

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Article

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

Biochimica et biophysica acta - 1 Aug 2013

Gonzalvez François, D'Aurelio Marilena, Boutant Marie, Moustapha Aoula, Puech Jean-Philippe, Landes Thomas, Arnauné-Pelloquin Laeticia, Vial Guillaume, Taleux Nellie, Slomianny Christian, Wanders Ronald J, Houtkooper Riekelt H, Bellenguer Pascale, Møller Ian Max, Gottlieb Eyal, Vaz Frederic M, Manfredi Giovanni, Petit Patrice X

Abstract excerpt

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...

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PMID: 23523468
DOI: 10.1016/j.bbadis.2013.03.005

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

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