Aberrant Schwann Cell Lipid Metabolism Linked to Mitochondrial Deficits Leads to Axon Degeneration and Neuropathy

@article{Viader2013AberrantSC,
  title={Aberrant Schwann Cell Lipid Metabolism Linked to Mitochondrial Deficits Leads to Axon Degeneration and Neuropathy},
  author={Andreu Viader and Yo Sasaki and Sungsu Kim and Amy M Strickland and Cayce S. Workman and Kui Yang and Richard W. Gross and Jeffrey D. Milbrandt},
  journal={Neuron},
  year={2013},
  volume={77},
  pages={886-898}
}
Mitochondrial dysfunction is a common cause of peripheral neuropathy. Much effort has been devoted to examining the role played by neuronal/axonal mitochondria, but how mitochondrial deficits in peripheral nerve glia (Schwann cells [SCs]) contribute to peripheral nerve diseases remains unclear. Here, we investigate a mouse model of peripheral neuropathy secondary to SC mitochondrial dysfunction (Tfam-SCKOs). We show that disruption of SC mitochondria activates a maladaptive integrated stress… CONTINUE READING
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