Protein zero (P0)–deficient mice show myelin degeneration in peripheral nerves characteristic of inherited human neuropathies

@article{Martini1995ProteinZ,
  title={Protein zero (P0)–deficient mice show myelin degeneration in peripheral nerves characteristic of inherited human neuropathies},
  author={Rudolf Martini and J{\"u}rgen Zielasek and Klaus Viktor Toyka and Karl Peter Giese and Melitta Schachner},
  journal={Nature Genetics},
  year={1995},
  volume={11},
  pages={281-286}
}
Mutations in the human gene for the myelin recognition molecule protein zero (P0) give rise to severe and progressive forms of dominantly inherited peripheral neuropathies. We have previously reported that mice homozygous for a null mutation in P0 have severely hypomyelinated nerves ten weeks after birth. Here we show hypomyelination already exists at day four with subsequent demyelination and impaired nerve conduction. Furthermore, heterozygous mutants show normal myelination, but develop… CONTINUE READING
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