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Titolo:
Normal neurogenesis and scrapie pathogenesis in neural grafts lacking the prion protein homologue Doppel
Autore:
Behrens, A; Brandner, S; Genoud, N; Aguzzi, A;
Indirizzi:
Univ Spital Zurich, Inst Neuropathol, CH-8091 Zurich, Switzerland Univ Spital Zurich Zurich Switzerland CH-8091 H-8091 Zurich, Switzerland
Titolo Testata:
EMBO REPORTS
fascicolo: 4, volume: 2, anno: 2001,
pagine: 347 - 352
SICI:
1469-221X(200104)2:4<347:NNASPI>2.0.ZU;2-S
Fonte:
ISI
Lingua:
ENG
Soggetto:
PURKINJE-CELL DEGENERATION; PRP-LIKE PROTEIN; MICE; GENE; EXPRESSION; SUSCEPTIBILITY; DEFICIENT; ATAXIA; BRAIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
24
Recensione:
Indirizzi per estratti:
Indirizzo: Aguzzi, A Univ Spital Zurich, Inst Neuropathol, Schmelzbergstr 12, CH-8091Zurich, Switzerland Univ Spital Zurich Schmelzbergstr 12 Zurich Switzerland CH-8091
Citazione:
A. Behrens et al., "Normal neurogenesis and scrapie pathogenesis in neural grafts lacking the prion protein homologue Doppel", EMBO REP, 2(4), 2001, pp. 347-352

Abstract

The agent that causes prion diseases is thought to be identical to PrPSc, a conformer of the normal prion protein PrPC. Recently a novel protein, termed Doppel (Dpl), was identified that shares significant biochemical and structural homology with PrPC. To investigate the function of Dpl in neurogenesis and in prion pathology, we generated embryonic stem (ES) cells harbouring a homozygous disruption of the Prnd gene that encodes Dpl. After in vitro differentiation and grafting into adult brains of PrPC-deficient Prnp(0/0) mice, Dpl-deficient ES cell-derived grafts contained all neural lineagesanalyzed, including neurons and astrocytes. When Prnd-deficient neural tissue was inoculated with scrapie prions, typical features of prion pathologyincluding spongiosis, gliosis and PrPSc accumulation, were observed. Therefore, Dpl is unlikely to exert a cell-autonomous function during neural differentiation and, in contrast to its homologue PrPC, is dispensable for prion disease progression and for generation of PrPSc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 18/01/20 alle ore 02:34:06