Molecular mechanisms of inherited demyelinating neuropathies.

  title={Molecular mechanisms of inherited demyelinating neuropathies.},
  author={Steven S. Scherer and Lawrence Wrabetz},
  volume={56 14},
The past 15 years have witnessed the identification of more than 25 genes responsible for inherited neuropathies in humans, many associated with primary alterations of the myelin sheath. A remarkable body of work in patients, as well as animal and cellular models, has defined the clinical and molecular genetics of these illnesses and shed light on how mutations in associated genes produce the heterogeneity of dysmyelinating and demyelinating phenotypes. Here, we review selected recent… CONTINUE READING