DNAJC19, a mitochondrial cochaperone associated with cardiomyopathy, forms a complex with prohibitins to regulate cardiolipin remodeling.

@article{RichterDennerlein2014DNAJC19AM,
  title={DNAJC19, a mitochondrial cochaperone associated with cardiomyopathy, forms a complex with prohibitins to regulate cardiolipin remodeling.},
  author={Ricarda Richter-Dennerlein and Anne Korwitz and Mathias Haag and Takashi Tatsuta and Sascha Dargazanli and M. J. Baker and Thorsten Decker and Tobias Lamkemeyer and Elena I Rugarli and Thomas Langer},
  journal={Cell metabolism},
  year={2014},
  volume={20 1},
  pages={158-71}
}
Prohibitins form large protein and lipid scaffolds in the inner membrane of mitochondria that are required for mitochondrial morphogenesis, neuronal survival, and normal lifespan. Here, we have defined the interactome of PHB2 in mitochondria and identified DNAJC19, mutated in dilated cardiomyopathy with ataxia, as binding partner of PHB complexes. We observed impaired cell growth, defective cristae morphogenesis, and similar transcriptional responses in the absence of either DNAJC19 or PHB2… CONTINUE READING
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