Abstract:
:Natural scrapie has been viewed both as a recessive trait and as a contagious disease modulated by a host locus. To address this conundrum, we determined the structure of the sheep prion protein (PrP) gene, which contains three exons and extends over 20 kb of DNA. In the United States 86.4% of scrapie cases occur in Suffolk sheep, and within this breed 49 +/- 6% (+/- S.D., n = 69) of healthy animals carry one or more PrP alleles encoding Arg (R)-171. Four scrapie-affected sheep were homozygous for wild-type PrP open reading frames encoding the alternative Gln (Q)-171 allele. Analysis of additional cases revealed that all were Q/Q-171 homozygotes (n = 31), yielding a probability of 0.000004 that PrP genotype is unrelated to susceptibility. These data imply that homozygosity for Q-171 codons is necessary but not sufficient for the development of natural scrapie, echo reports of recessive manifestation, and parallel over-representation of PRNP codon 129 homozygotes in Creutzfeldt-Jakob disease of humans. Whereas progress has been substantial regarding experimental scrapie in rodents, the occurrence and spread of disease in flocks of sheep has remained enigmatic. Appreciation of the relationship between codon 171 genotype and susceptibility may help define the molecular basis of natural scrapie.
journal_name
Genes Devjournal_title
Genes & developmentauthors
Westaway D,Zuliani V,Cooper CM,Da Costa M,Neuman S,Jenny AL,Detwiler L,Prusiner SBdoi
10.1101/gad.8.8.959subject
Has Abstractpub_date
1994-04-15 00:00:00pages
959-69issue
8eissn
0890-9369issn
1549-5477journal_volume
8pub_type
杂志文章abstract::The number of known long noncoding RNA (lncRNA) functions is rapidly growing, but how those functions are encoded in their sequence and structure remains poorly understood. NORAD (noncoding RNA activated by DNA damage) is a recently characterized, abundant, and highly conserved lncRNA that is required for proper mitot...
journal_title:Genes & development
pub_type: 杂志文章
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journal_title:Genes & development
pub_type: 杂志文章
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journal_title:Genes & development
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journal_title:Genes & development
pub_type: 杂志文章
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journal_title:Genes & development
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journal_title:Genes & development
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journal_title:Genes & development
pub_type: 杂志文章
doi:10.1101/gad.13.22.2934
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journal_title:Genes & development
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journal_title:Genes & development
pub_type: 杂志文章
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更新日期:2002-07-01 00:00:00
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journal_title:Genes & development
pub_type: 杂志文章
doi:10.1101/gad.3.5.687
更新日期:1989-05-01 00:00:00
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journal_title:Genes & development
pub_type: 杂志文章,评审
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更新日期:2010-06-15 00:00:00
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journal_title:Genes & development
pub_type: 杂志文章
doi:10.1101/gad.4.10.1650
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journal_title:Genes & development
pub_type: 杂志文章
doi:10.1101/gad.5.7.1136
更新日期:1991-07-01 00:00:00
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journal_title:Genes & development
pub_type: 杂志文章
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journal_title:Genes & development
pub_type: 杂志文章
doi:10.1101/gad.326314.119
更新日期:2019-10-01 00:00:00
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journal_title:Genes & development
pub_type: 杂志文章
doi:10.1101/gad.11.11.1422
更新日期:1997-06-01 00:00:00
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journal_title:Genes & development
pub_type: 杂志文章
doi:10.1101/gad.10.12.1455
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journal_title:Genes & development
pub_type: 杂志文章
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journal_title:Genes & development
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journal_title:Genes & development
pub_type: 杂志文章
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journal_title:Genes & development
pub_type: 杂志文章
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journal_title:Genes & development
pub_type: 杂志文章
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更新日期:2011-04-15 00:00:00
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journal_title:Genes & development
pub_type: 杂志文章,评审
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更新日期:2004-08-15 00:00:00
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journal_title:Genes & development
pub_type: 杂志文章
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journal_title:Genes & development
pub_type: 杂志文章
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更新日期:1987-04-01 00:00:00
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journal_title:Genes & development
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更新日期:2008-01-01 00:00:00