RGD Reference Report - Genes contributing to prion pathogenesis. - Rat Genome Database

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Genes contributing to prion pathogenesis.

Authors: Tamgüney, Gültekin  Giles, Kurt  Glidden, David V  Lessard, Pierre  Wille, Holger  Tremblay, Patrick  Groth, Darlene F  Yehiely, Fruma  Korth, Carsten  Moore, Richard C  Tatzelt, Jörg  Rubinstein, Eric  Boucheix, Claude  Yang, Xiaoping  Stanley, Pamela  Lisanti, Michael P  Dwek, Raymond A  Rudd, Pauline M  Moskovitz, Jackob  Epstein, Charles J  Cruz, Tracey Dawson  Kuziel, William A  Maeda, Nobuyo  Sap, Jan  Ashe, Karen Hsiao  Carlson, George A  Tesseur, Ina  Wyss-Coray, Tony  Mucke, Lennart  Weisgraber, Karl H  Mahley, Robert W  Cohen, Fred E  Prusiner, Stanley B 
Citation: Tamgüney G, etal., J Gen Virol. 2008 Jul;89(Pt 7):1777-88. doi: 10.1099/vir.0.2008/001255-0.
RGD ID: 13782161
Pubmed: PMID:18559949   (View Abstract at PubMed)
PMCID: PMC2828448   (View Article at PubMed Central)
DOI: DOI:10.1099/vir.0.2008/001255-0   (Journal Full-text)

Prion diseases are caused by conversion of a normally folded, non-pathogenic isoform of the prion protein (PrP(C)) to a misfolded, pathogenic isoform (PrP(Sc)). Prion inoculation experiments in mice expressing homologous PrP(C) molecules on different genetic backgrounds displayed different incubation times, indicating that the conversion reaction may be influenced by other gene products. To identify genes that contribute to prion pathogenesis, we analysed incubation times of prions in mice in which the gene product was inactivated, knocked out or overexpressed. We tested 20 candidate genes, because their products either colocalize with PrP, are associated with Alzheimer's disease, are elevated during prion disease, or function in PrP-mediated signalling, PrP glycosylation, or protein maintenance. Whereas some of the candidates tested may have a role in the normal function of PrP(C), our data show that many genes previously implicated in prion replication have no discernible effect on the pathogenesis of prion disease. While most genes tested did not significantly affect survival times, ablation of the amyloid beta (A4) precursor protein (App) or interleukin-1 receptor, type I (Il1r1), and transgenic overexpression of human superoxide dismutase 1 (SOD1) prolonged incubation times by 13, 16 and 19 %, respectively.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
prion disease disease_progressionIMP 13782161 RGD 
prion disease disease_progressionISOSOD1 (Homo sapiens)13782161; 13782161 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Sod1  (superoxide dismutase 1)

Genes (Mus musculus)
Sod1  (superoxide dismutase 1, soluble)

Genes (Homo sapiens)
SOD1  (superoxide dismutase 1)


Additional Information