Loss of protein association causes cardiolipin degradation in Barth syndrome

@inproceedings{Xu2016LossOP,
  title={Loss of protein association causes cardiolipin degradation in Barth syndrome},
  author={Yang Xu and Colin K L Phoon and Bob Berno and Kenneth D'Souza and Esthelle Hoedt and Guoan Zhang and Thomas A Neubert and Richard M Epand and Mindong Ren and Michael Schlame},
  booktitle={Nature chemical biology},
  year={2016}
}
Cardiolipin is a specific mitochondrial phospholipid that has a high affinity for proteins and that stabilizes the assembly of supercomplexes involved in oxidative phosphorylation. We found that sequestration of cardiolipin in protein complexes is critical to protect it from degradation. The turnover of cardiolipin is slower by almost an order of magnitude than the turnover of other phospholipids. However, in subjects with Barth syndrome, cardiolipin is rapidly degraded via the intermediate… CONTINUE READING
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