Article
Doppel-induced cerebellar degeneration in transgenic mice.
Proceedings of the National Academy of Sciences of the United States of America - 18 Dec 2001
Moore R C, Mastrangelo P, Bouzamondo E, Heinrich C, Legname G, Prusiner S B, Hood L, Westaway D, DeArmond S J, Tremblay P
Abstract excerpt
Doppel (Dpl) is a paralog of the mammalian prion protein (PrP); it is abundant in testes but expressed at low levels in the adult central nervous system. In two Prnp-deficient (Prnp(0/0)) mouse lines (Ngsk and Rcm0), Dpl overexpression correlated with ataxia and death of cerebellar neurons. To determine whether Dpl overexpression, rather than the dysregulation of genes neighboring the Prn gene complex, was...
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