Petit 2017 MiP2017
Petit PX (2017)
We believe that the link between ROS production and sarcomere dysorganization could be straightforward. But, there are some clues linked with autophagic flux destabilization which might be involved in the pathophysiology of the disease. Indeed, after the discovery of inhibition the mitochondrially amplified Fas and TNFα apoptosis, we have highlighted a cardiolipin/caspase-8/Bid activation platform at the mitochondrial outer membrane and investigated the occurrence of autophagic processes. The main autophagic processes are unaffected, whereas mitophagy which would have been useful to eliminate the dysfunctionnal mitochondria is totally abolished. Our results are discussed in light of the recent results, especially, the elegant work of Wang’s team, that bridges the gap between abnormal ROS production in BTHS derived-iPSCs and the mechanical defect of the sarcomeric structure and function.
• Bioblast editor: Kandolf G
Labels: Pathology: Other
- CNRS and INSERM U1124, Univ Paris-Descartes, Centre Univ Saints-Pères, Paris, France. -
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