Scripps VIVO scripps research logo

  • Index
  • Log in
  • Home
  • People
  • Organizations
  • Research
  • Events
Search form
As of April 1st VIVO Scientific Profiles will no longer updated for faculty, and the link to VIVO will be removed from the library website. Faculty profile pages will continue to be updated via Interfolio. VIVO will continue being used behind the scenes to update graduate student profiles. Please contact helplib@scripps.edu if you have questions.
How to download citations from VIVO | Alternative profile options

Onset of ataxia and purkinje cell loss in prp null mice inversely correlated with dpl level in brain

Academic Article
uri icon
  • Overview
  • Research
  • Identity
  • Additional Document Info
  • View All
scroll to property group menus

Overview

authors

  • Rossi, D.
  • Cozzio, A.
  • Flechsig, E.
  • Klein, M. A.
  • Rulicke, T.
  • Aguzzi, A.
  • Weissmann, Charles

publication date

  • February 2001

journal

  • EMBO Journal  Journal

abstract

  • PrP knockout mice in which only the open reading frame was disrupted ('Zürich 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, 'Zürich II', with a 2.9 kb PRNP: deletion, developed this phenotype at approximately 10 months (50% morbidity). A single PRNP: allele abolished the syndrome. Compound Zürich I/Zürich II heterozygotes had half the Dpl of Zürich II mice and developed symptoms 6 months later. Zürich II mice transgenic for a PRND:-containing cosmid expressed Dpl at twice the level and became ataxic approximately 5 months earlier. Thus, Dpl levels in brain and onset of the ataxic syndrome are inversely correlated.
  • PrP knockout mice in which only the open reading frame was disrupted ('Z�rich 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, 'Z�rich II', with a 2.9 kb PRNP: deletion, developed this phenotype at approximately 10 months (50% morbidity). A single PRNP: allele abolished the syndrome. Compound Z�rich I/Z�rich II heterozygotes had half the Dpl of Z�rich II mice and developed symptoms 6 months later. Z�rich II mice transgenic for a PRND:-containing cosmid expressed Dpl at twice the level and became ataxic approximately 5 months earlier. Thus, Dpl levels in brain and onset of the ataxic syndrome are inversely correlated.

subject areas

  • Alleles
  • Animals
  • Ataxia
  • Base Sequence
  • Brain
  • Cosmids
  • DNA Primers
  • GPI-Linked Proteins
  • Immunoblotting
  • Immunohistochemistry
  • Mice
  • Mice, Knockout
  • Multigene Family
  • Open Reading Frames
  • Phenotype
  • Prions
  • Purkinje Cells
  • RNA, Messenger
  • Transgenes
scroll to property group menus

Research

keywords

  • cerebellar syndrome
  • doppel protein
  • gene deletion
  • granule cells
  • prion protein
scroll to property group menus

Identity

PubMed Central ID

  • PMC145426

International Standard Serial Number (ISSN)

  • 0261-4189

Digital Object Identifier (DOI)

  • 10.1093/emboj/20.4.694

PubMed ID

  • 11179214
scroll to property group menus

Additional Document Info

start page

  • 694

end page

  • 702

volume

  • 20

issue

  • 4

©2022 The Scripps Research Institute | Terms of Use | Powered by VIVO

  • About
  • Contact Us
  • Support