Imbalanced OPA1 processing and mitochondrial fragmentation cause heart failure in mice

@article{Wai2015ImbalancedOP,
  title={Imbalanced OPA1 processing and mitochondrial fragmentation cause heart failure in mice},
  author={Timothy Wai and Jaime Garc{\'i}a-Prieto and Michael J. Baker and Carsten Merkwirth and Paule B{\'e}nit and Pierre Rustin and Francisco Javier Rup{\'e}rez and Coral Barbas and Borja Ib{\'a}{\~n}ez and Thomas Langer},
  journal={Science},
  year={2015},
  volume={350}
}
Mitochondrial morphology is shaped by fusion and division of their membranes. Here, we found that adult myocardial function depends on balanced mitochondrial fusion and fission, maintained by processing of the dynamin-like guanosine triphosphatase OPA1 by the mitochondrial peptidases YME1L and OMA1. Cardiac-specific ablation of Yme1l in mice activated OMA1 and accelerated OPA1 proteolysis, which triggered mitochondrial fragmentation and altered cardiac metabolism. This caused dilated… CONTINUE READING
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