ArticleMolecular and cellular biology1988
Genetics and polymorphism of the mouse prion gene complex: control of scrapie incubation time.
Article in Molecular and cellular biology, 1988. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 67 papers.
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Who cites it
67 citing papers in PubMed.
- The L108I polymorphism in mouse prion protein drives spontaneous disease and enhances transmission of atypical and classical prion strains.Brain pathology (Zurich, Switzerland) · 2026Article
- The Evolution of Experimental Rodent Models for Prion Diseases.Journal of neurochemistry · 2025Review
- How an Infection of Sheep Revealed Prion Mechanisms in Alzheimer's Disease and Other Neurodegenerative Disorders.International journal of molecular sciences · 2021Review
- Kinetics of Human Mutant Tau Prion Formation in the Brains of 2 Transgenic Mouse Lines.JAMA neurology · 2017Article
- Neurodegenerative Disease Transmission and Transgenesis in Mice.Cold Spring Harbor perspectives in biology · 2017Review
- Amyloid fibrils from the N-terminal prion protein fragment are infectious.Proceedings of the National Academy of Sciences of the United States of America · 2016Article
- Guinea Pig Prion Protein Supports Rapid Propagation of Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease Prions.Journal of virology · 2016Article
- Prions are affected by evolution at two levels.Cellular and molecular life sciences : CMLS · 2016Review
- Evidence that bank vole PrP is a universal acceptor for prions.PLoS pathogens · 2014Article
- Biaryl amides and hydrazones as therapeutics for prion disease in transgenic mice.The Journal of pharmacology and experimental therapeutics · 2013Article
- Mouse prion protein polymorphism Phe-108/Val-189 affects the kinetics of fibril formation and the response to seeding: evidence for a two-step nucleation polymerization mechanism.The Journal of biological chemistry · 2013Article
- Chimeric elk/mouse prion proteins in transgenic mice.The Journal of general virology · 2013Article
- Article
- Overexpression of the Hspa13 (Stch) gene reduces prion disease incubation time in mice.Proceedings of the National Academy of Sciences of the United States of America · 2012Article
- Spontaneous generation of rapidly transmissible prions in transgenic mice expressing wild-type bank vole prion protein.Proceedings of the National Academy of Sciences of the United States of America · 2012Article
- Protease-resistant prions selectively decrease Shadoo protein.PLoS pathogens · 2011Article
- Down-regulation of Shadoo in prion infections traces a pre-clinical event inversely related to PrP(Sc) accumulation.PLoS pathogens · 2011Article
- Dominant prion mutants induce curing through pathways that promote chaperone-mediated disaggregation.Nature structural & molecular biology · 2011Article
- Sex effects in mouse prion disease incubation time.PloS one · 2011Article
- Article
7 more citing papers are in PubMed but not listed here.
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8 authors.
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Abstract
The mouse prion protein (PrP) gene (Prn-p), which encodes the only macromolecule that has been identified in scrapie prions, is tightly linked or identical to a gene (Prn-i) that controls the duration of the scrapie incubation period in mice. Constellations of restriction fragment length polymorphisms distinguish haplotypes a to f of Prn-p. The Prn-pb allele encodes a PrP that differs in sequence from those encoded by the other haplotypes and, in inbred mouse strains, correlates with long scrapie incubation time (Westaway et al., Cell 51: 651-662, 1987). In segregating crosses of mice, we identified rare individuals with a divergent scrapie incubation time phenotype and Prn-p genotype, but progeny testing to demonstrate meiotic recombination was not possible because scrapie is a lethal disease. Crosses involving the a, d, and e haplotypes demonstrated that genes unlinked to Prn-p could modulate scrapie incubation time and that there were only two alleles of Prn-i among the mouse strains tested. All inbred strains of mice that had the Prnb haplotype were probably direct descendants of the I/LnJ progenitors. We established the linkage relationship between the prion gene complex (Prn) and other chromosome 2 genes; the gene order, proximal to distal, is B2m-II-1a-Prn-Itp-A. Recombination suppression in the B2m-Prn-p interval occurred during the crosses involved in transferring the I/LnJ Prnb complex into a C57BL/6J background. Transmission ratio distortion by Prna/Prnb heterozygous males was also observed in the same crosses. These phenomena, together with the founder effect, would favor apparent linkage disequilibrium between Prn-p and Prn-i. Therefore, transmission genetics may underestimate the number of genes in Prn.
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