| First Author | Nishida N | Year | 1999 |
| Journal | Lab Invest | Volume | 79 |
| Issue | 6 | Pages | 689-97 |
| PubMed ID | 10378511 | Mgi Jnum | J:55709 |
| Mgi Id | MGI:1339235 | Citation | Nishida N, et al. (1999) A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination. Lab Invest 79(6):689-97 |
| abstractText | Disruption of both alleles of the prion protein gene, Prnp, renders mice resistant to prions; in a Prnp o/o line reported by some of us, mice progressively developed ataxia and Purkinje cell loss. Here we report torpedo-like axonal swellings associated with residual Purkinje cells in Prnp o/o mice, and we demonstrate abnormal myelination in the spinal cord and peripheral nerves in mice from two independently established Prnp o/o lines. Mice were successfully rescued from both demyelination and Purkinje cell degeneration by introduction of a transgene encoding wild-type mouse cellular prion protein. These findings suggest that cellular prion protein expression may be necessary to maintain the integrity of the nervous system. |
