Article
A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination.
Laboratory investigation; a journal of technical methods and pathology - 1 Jun 1999
Nishida N, Tremblay P, Sugimoto T, Shigematsu K, Shirabe S, Petromilli C, Erpel S P, Nakaoke R, Atarashi R, Houtani T, Torchia M, Sakaguchi S, DeArmond S J, Prusiner S B, Katamine S
Abstract excerpt
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...
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