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

作者：Nishida N; Tremblay P; Sugimoto T; Shigematsu K; Shirabe S; Petromilli C; Erpel SP; Nakaoke R; Atarashi R; Houtani T; Torchia M; Sakaguchi S; DeArmond SJ; Prusiner SB; Katamine S^*

来源：Laboratory Investigation, 1999, 79(6): 689-697.

Disruption of both alleles of the prion protein gene, Pmp, 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.

• 出版日期1999-6