Animal spongiform encephalopathies--an update. Part 1. Scrapie and lesser known animal spongiform encephalopathies. 1994

B E Schreuder
DLO-Central Veterinary Institute, Lelystad, The Netherlands.

The present article (part I) reviews recent developments in animal spongiform encephalopathies (SEs), with the exception of bovine spongiform encephalopathy (BSE), which is dealt with in part II. The article focuses on scrapie and describes epidemiological aspects and the prospects for a preclinical diagnosis. Up to now, confirmatory diagnosis of scrapie depended on histological examination of the brain, collected during post-mortem examination from sheep with clinical signs of the disease. An altered protein, PrPSc, can be detected in the brain of diseased animals. The demonstration of the same protein in the spleen and in peripheral lymph nodes of infected animals seems to offer interesting possibilities of arriving at a method for a preclinical diagnosis, and thus a diagnosis in the live animal. Progress has also been made in our understanding of the relationship between the genetic constitution and susceptibility of the host. Susceptibility is expressed as the survival time of sheep inoculated with scrapie. This was thought to be determined by a single genetic locus designated the Sip gene (scrapie incubation period gene). Putative markers for the two alleles of the Sip gene, sA and pA, have been discovered, consisting of restriction fragment length polymorphisms (RFLPs). In field tests, however, the link between these markers and the length of incubation time was far from consistent. These RFLPs were found to be situated outside the prion-protein-coding region of the ovine gene. In later studies, RFLPs were detected inside this region. These markers appear to be more informative, i.e. they correspond with a difference in the length of the scrapie incubation period.(ABSTRACT TRUNCATED AT 250 WORDS)

UI MeSH Term Description Entries
D008907 Mink Carnivores of genera Mustela and Neovison of the family MUSTELIDAE. The European mink has white upper and lower lips while the American mink lacks white upper lip. American Mink,European Mink,Mustela lutreola,Mustela macrodon,Mustela vison,Neovison vison,Sea Mink,Mink, American,Mink, European,Mink, Sea,Minks,Minks, Sea,Sea Minks,vison, Neovison
D011328 Prions Small proteinaceous infectious particles which resist inactivation by procedures that modify NUCLEIC ACIDS and contain an abnormal isoform of a cellular protein which is a major and necessary component. The abnormal (scrapie) isoform is PrPSc (PRPSC PROTEINS) and the cellular isoform PrPC (PRPC PROTEINS). The primary amino acid sequence of the two isoforms is identical. Human diseases caused by prions include CREUTZFELDT-JAKOB SYNDROME; GERSTMANN-STRAUSSLER SYNDROME; and INSOMNIA, FATAL FAMILIAL. Mink Encephalopathy Virus,Prion,Encephalopathy Virus, Mink
D002371 Cat Diseases Diseases of the domestic cat (Felis catus or F. domesticus). This term does not include diseases of the so-called big cats such as CHEETAHS; LIONS; tigers, cougars, panthers, leopards, and other Felidae for which the heading CARNIVORA is used. Feline Diseases,Cat Disease,Disease, Cat,Disease, Feline,Diseases, Cat,Diseases, Feline,Feline Disease
D002415 Cats The domestic cat, Felis catus, of the carnivore family FELIDAE, comprising over 30 different breeds. The domestic cat is descended primarily from the wild cat of Africa and extreme southwestern Asia. Though probably present in towns in Palestine as long ago as 7000 years, actual domestication occurred in Egypt about 4000 years ago. (From Walker's Mammals of the World, 6th ed, p801) Felis catus,Felis domesticus,Domestic Cats,Felis domestica,Felis sylvestris catus,Cat,Cat, Domestic,Cats, Domestic,Domestic Cat
D000818 Animals Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA. Animal,Metazoa,Animalia
D000835 Animals, Wild Animals considered to be wild or feral or not adapted for domestic use. It does not include wild animals in zoos for which ANIMALS, ZOO is available. Animals, Nondomestic,Animals, Nondomesticated,Animals, Feral,Stray Animals,Animal, Feral,Animal, Nondomestic,Animal, Nondomesticated,Animal, Stray,Animal, Wild,Animals, Stray,Feral Animal,Feral Animals,Nondomestic Animal,Nondomestic Animals,Nondomesticated Animal,Nondomesticated Animals,Stray Animal,Wild Animal,Wild Animals
D000836 Animals, Zoo Animal population groups or individual animals that reside in captivity at a zoological park. Animal, Zoo,Zoo Animal,Zoo Animals
D012608 Scrapie A fatal disease of the nervous system in sheep and goats, characterized by pruritus, debility, and locomotor incoordination. It is caused by proteinaceous infectious particles called PRIONS. Rida
D012756 Sheep Any of the ruminant mammals with curved horns in the genus Ovis, family Bovidae. They possess lachrymal grooves and interdigital glands, which are absent in GOATS. Ovis,Sheep, Dall,Dall Sheep,Ovis dalli

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