Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia

PrP knockout mice with disruption of only the PrP-encoding region (Zürich I-type) remain healthy, whereas mice with deletions extending upstream of the PrP-encoding exon (Nagasaki-type) suffer Purkinje cell loss and ataxia, associated with ectopic expression of Doppel in brain, particularly in Purki...

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المؤلفون الرئيسيون: Flechsig, Eckhard, Hegyi, Ivan, Leimeroth, Rainer, Zuniga, Armando, Rossi, Daniela, Cozzio, Antonio, Schwarz, Petra, Rülicke, Thomas, Götz, Jürgen, Aguzzi, Adriano, Weissmann, Charles
التنسيق: مقال
اللغة:en
منشور في: Oxford University Press 2003
الموضوعات:
Articles
الوصول للمادة أونلاين:https://ncbi.nlm.nih.gov/pmc/articles/PMC162137/
https://ncbi.nlm.nih.gov/pubmed/12805223
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/emboj/cdg285
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spelling pubmed-1621372003-07-10 Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia Flechsig, Eckhard Hegyi, Ivan Leimeroth, Rainer Zuniga, Armando Rossi, Daniela Cozzio, Antonio Schwarz, Petra Rülicke, Thomas Götz, Jürgen Aguzzi, Adriano Weissmann, Charles EMBO J Articles PrP knockout mice with disruption of only the PrP-encoding region (Zürich I-type) remain healthy, whereas mice with deletions extending upstream of the PrP-encoding exon (Nagasaki-type) suffer Purkinje cell loss and ataxia, associated with ectopic expression of Doppel in brain, particularly in Purkinje cells. The phenotype is abrogated by co-expression of full-length PrP. Doppel is 25% similar to PrP, has the same globular fold, but lacks the flexible N-terminal tail. We now show that in Zürich I-type PrP-null mice, expression of N-terminally truncated PrP targeted to Purkinje cells also leads to Purkinje cell loss and ataxia, which are reversed by PrP. Doppel and truncated PrP probably cause Purkinje cell degeneration by the same mechanism. Oxford University Press 2003-06-16 /pmc/articles/PMC162137/ /pubmed/12805223 http://dx.doi.org/10.1093/emboj/cdg285 Text en Copyright © 2003 European Molecular Biology Organization
institution US National Library of Medicine
collection PubMed Central
language en
format Article
topic Articles
spellingShingle Articles
Flechsig, Eckhard
Hegyi, Ivan
Leimeroth, Rainer
Zuniga, Armando
Rossi, Daniela
Cozzio, Antonio
Schwarz, Petra
Rülicke, Thomas
Götz, Jürgen
Aguzzi, Adriano
Weissmann, Charles
Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia
description PrP knockout mice with disruption of only the PrP-encoding region (Zürich I-type) remain healthy, whereas mice with deletions extending upstream of the PrP-encoding exon (Nagasaki-type) suffer Purkinje cell loss and ataxia, associated with ectopic expression of Doppel in brain, particularly in Purkinje cells. The phenotype is abrogated by co-expression of full-length PrP. Doppel is 25% similar to PrP, has the same globular fold, but lacks the flexible N-terminal tail. We now show that in Zürich I-type PrP-null mice, expression of N-terminally truncated PrP targeted to Purkinje cells also leads to Purkinje cell loss and ataxia, which are reversed by PrP. Doppel and truncated PrP probably cause Purkinje cell degeneration by the same mechanism.
author Flechsig, Eckhard
Hegyi, Ivan
Leimeroth, Rainer
Zuniga, Armando
Rossi, Daniela
Cozzio, Antonio
Schwarz, Petra
Rülicke, Thomas
Götz, Jürgen
Aguzzi, Adriano
Weissmann, Charles
author_facet Flechsig, Eckhard
Hegyi, Ivan
Leimeroth, Rainer
Zuniga, Armando
Rossi, Daniela
Cozzio, Antonio
Schwarz, Petra
Rülicke, Thomas
Götz, Jürgen
Aguzzi, Adriano
Weissmann, Charles
author_sort Flechsig, Eckhard
title Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia
title_short Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia
title_full Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia
title_fullStr Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia
title_full_unstemmed Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia
title_sort expression of truncated prp targeted to purkinje cells of prp knockout mice causes purkinje cell death and ataxia
publisher Oxford University Press
publisher_facet Oxford University Press
publishDate 2003
url https://ncbi.nlm.nih.gov/pmc/articles/PMC162137/
https://ncbi.nlm.nih.gov/pubmed/12805223
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/emboj/cdg285
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