Mitochondrial quality control: a matter of life and death for neurons.

  title={Mitochondrial quality control: a matter of life and death for neurons.},
  author={Elena I Rugarli and Thomas Langer},
  journal={The EMBO journal},
  volume={31 6},
Neuronal survival critically depends on the integrity and functionality of mitochondria. A hierarchical system of cellular surveillance mechanisms protects mitochondria against stress, monitors mitochondrial damage and ensures the selective removal of dysfunctional mitochondrial proteins or organelles. Mitochondrial proteases emerge as central regulators that coordinate different quality control (QC) pathways within an interconnected network of mechanisms. A failure of this system causes… CONTINUE READING
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Whole - exome sequencing identifies homozygous AFG 3 L 2 mutations in a spastic ataxianeuropathy syndrome linked to mitochondrial mAAA proteases

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