Article
Heterozygous peripheral myelin protein 22-deficient mice are affected by a progressive demyelinating tomaculous neuropathy.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 15 Jun 1997
Adlkofer K, Frei R, Neuberg D H, Zielasek J, Toyka K V, Suter U
Abstract excerpt
Hereditary neuropathy with liability to pressure palsy (HNPP) is associated with a heterozygous 1.5 megabase deletion on chromosome 17 that includes the peripheral myelin protein (PMP) gene PMP22. We show that heterozygous PMP22 knock-out mice, which carry only one functional pmp22 allele and thus genetically mimic HNPP closely, display similar morphological and electrophysiological features as observed in HNPP...
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