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Article

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

Proceedings of the National Academy of Sciences of the United States of America - 9 Mar 2004

Anderson Lucy, Rossi Daniela, Linehan Jackie, Brandner Sebastian, Weissmann Charles

Abstract excerpt

The Doppel (Dpl) and Prion (PrP) proteins show 25% sequence identity and share several structural features with only minor differences. Dpl shows a PrP-like fold of its C-terminal globular domain and lacks the flexible N-terminal tail. The physiological functions of both proteins are unknown. However, ubiquitous Dpl overexpression in the brain of PrP knockout mice correlated with ataxia and Purkinje cell...

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Identifiers

PMID: 15007176
DOI: 10.1073/pnas.0308681101
PMCID: PMC373516

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Transgene-driven expression of the Doppel protein in Purkinje cells causes Purkinje cell degeneration and motor impairment.PMID 15007176Read ↗

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

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

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

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

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

Doppel induces autophagic stress in prion protein-deficient Purkinje cells.Semantic proximity Read ↗

Doppel induces degeneration of cerebellar Purkinje cells independently of Bax.Semantic proximity Read ↗

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

Genetic mapping of activity determinants within cellular prion proteins: N-terminal modules in PrPC offset pro-apoptotic activity of the Doppel helix B/B' region.Semantic proximity Read ↗

Normal neurogenesis and scrapie pathogenesis in neural grafts lacking the prion protein homologue Doppel.Semantic proximity Read ↗

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