A mouse prion protein transgene rescues mice deficient for the prion protein gene from Purkinje cell degeneration and demyelination

摘要: Disruption of both alleles the prion protein gene, Prnp, renders mice resistant to prions; in a Prnp o/o line reported by some us, progressively developed ataxia and Purkinje cell loss. Here we report torpedo-like axonal swellings associated with residual cells mice, demonstrate abnormal myelination spinal cord peripheral nerves from two independently established lines. Mice were successfully rescued demyelination degeneration introduction transgene encoding wild-type mouse cellular protein. These findings suggest that expression may be necessary maintain integrity nervous system.