期刊
EMBO JOURNAL
卷 20, 期 4, 页码 694-702出版社
WILEY
DOI: 10.1093/emboj/20.4.694
关键词
cerebellar syndrome; doppel protein; gene deletion; granule cells; prion protein
PrP knockout mice in which only the open reading frame was disrupted ('Zurich I') remained healthy. However, more extensive deletions resulted in ataxia, Purkinje cell loss and ectopic expression in brain of Doppel (Dpl), encoded by the downstream gene, Prnd. A new PrP knockout line,'Zurich II', with a 2.9 kb Prnp deletion, developed this phenotype at similar to 10 months (50% morbidity), A single Prnp allele abolished the syndrome. Compound Zurich I/Zurich II heterozygotes had half the Dpl of Zurich II mice and developed symptoms 6 months later. Zurich II mice transgenic for a Prnd-containing cosmid expressed Dpl at twice the level and became ataxic similar to5 months earlier. Thus, Dpl levels in brain and onset of the ataxic syndrome are inversely correlated.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据