Send to:

Choose Destination
See comment in PubMed Commons below
Neuron. 2008 Feb 7;57(3):329-30. doi: 10.1016/j.neuron.2008.01.017.

Balancing between adaptive and maladaptive cellular stress responses in peripheral neuropathy.

Author information

  • 1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA.


Point mutations in "myelin genes" result in a spectrum of inherited demyelinating neuropathies. The understanding of the pathomechanisms by which these mutations produce phenotypes remains limited. In this issue of Neuron, Wrabetz and colleagues report that the unfolded protein response (UPR) is responsible for demyelination in a Charcot-Marie-Tooth disease type 1B (CMT1B) mouse model. Deletion of the UPR mediator transcription factor CHOP completely rescues the motor deficit and ameliorates the neuropathy phenotype.

[PubMed - indexed for MEDLINE]
Free full text
PubMed Commons home

PubMed Commons

How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science
    Loading ...
    Write to the Help Desk