Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

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

The EMBO journal - 16 Jun 2003

Flechsig Eckhard, Hegyi Ivan, Leimeroth Rainer, Zuniga Armando, Rossi Daniela, Cozzio Antonio, Schwarz Petra, Rülicke Thomas, Götz Jürgen, Aguzzi Adriano, Weissmann Charles

Abstract excerpt

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...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Ataxia
Share to Brain

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Share to Cell Death

Share to GPI-Linked Proteins

Share to Immunohistochemistry

Share to Mice, Transgenic

Share to Prions

Share to Purkinje Cells

Source

Open in PubMed Open full text

Identifiers

PMID: 12805223
DOI: 10.1093/emboj/cdg285
PMCID: PMC162137

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia.PMID 12805223Read ↗

Transgene-driven expression of the Doppel protein in Purkinje cells causes Purkinje cell degeneration and motor impairment.Semantic proximity Read ↗

Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brain.Semantic proximity Read ↗

Doppel-induced Purkinje cell death is stoichiometrically abrogated by prion protein.Semantic proximity Read ↗

Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP geneSemantic proximity Read ↗

Doppel-induced cerebellar degeneration in transgenic mice.Semantic proximity Read ↗

Deletion of N-terminal residues 23-88 from prion protein (PrP) abrogates the potential to rescue PrP-deficient mice from PrP-like protein/doppel-induced Neurodegeneration.Semantic proximity Read ↗

Truncated prion protein and Doppel are myelinotoxic in the absence of oligodendrocytic PrPC.Semantic proximity Read ↗

A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination.Semantic proximity Read ↗

The prion gene complex encoding PrP(C) and Doppel: insights from mutational analysis.Semantic proximity Read ↗

Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions.Semantic proximity Read ↗

Autophagy and cell death of Purkinje cells overexpressing Doppel in Ngsk Prnp-deficient mice.Semantic proximity Read ↗

The toppler mouse: a novel mutant exhibiting loss of Purkinje cells.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.