Biomaterial Database

Introduction to the transmissible spongiform encephalopathies or prion diseases. 2003

Bruce Chesebro

Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institutes of Allergy and Infectious Diseases, Hamilton, Montana 59840, USA.

Sheep scrapie has been known for at least 200 years and was described as a transmissible disease over 100 years ago. Since then, three groups of transmissible spongiform encephalopathies or TSE diseases have been identified in humans including familial, infectious and sporadic types. The discovery of the prion protein (PrP) in the 1980s greatly accelerated knowledge of the biology and pathogenesis of TSE diseases as this protein was found to play a critical role in disease susceptibility and the TSE species-barrier and may also be a component of the infectious agent itself. Nevertheless, the nature of the TSE agents remains an enigma. Proof of the protein-only hypothesis may require generation of biologically active transmissible agent in a cell-free environment where a virus cannot replicate. Conversely, proof of a viral aetiology will require identification and isolation of a candidate virus. Further understanding of the structure of the disease-associated protease-resistant PrP should help elucidate the mechanism of PrP conversion from the normal to the abnormal form. Such information should open up new approaches to both diagnosis and therapy.

UI	MeSH Term	Description	Entries
D007562	Creutzfeldt-Jakob Syndrome	A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS. (From N Engl J Med, 1998 Dec 31;339(27))	New Variant Creutzfeldt-Jakob Disease,Spongiform Encephalopathy, Subacute,CJD (Creutzfeldt-Jakob Disease),Creutzfeldt Jacob Disease,Creutzfeldt-Jakob Disease,Creutzfeldt-Jakob Disease, Familial,Creutzfeldt-Jakob Disease, New Variant,Creutzfeldt-Jakob Disease, Variant,Familial Creutzfeldt-Jakob Disease,Jakob-Creutzfeldt Disease,Jakob-Creutzfeldt Syndrome,V-CJD (Variant-Creutzfeldt-Jakob Disease),Variant Creutzfeldt-Jakob Disease,CJD (Creutzfeldt Jakob Disease),Creutzfeldt Jakob Disease,Creutzfeldt Jakob Disease, Familial,Creutzfeldt Jakob Disease, New Variant,Creutzfeldt Jakob Disease, Variant,Creutzfeldt Jakob Syndrome,Creutzfeldt-Jakob Diseases, Familial,Disease, Creutzfeldt Jacob,Disease, Creutzfeldt-Jakob,Disease, Familial Creutzfeldt-Jakob,Disease, Jakob-Creutzfeldt,Encephalopathies, Subacute Spongiform,Encephalopathy, Subacute Spongiform,Familial Creutzfeldt Jakob Disease,Familial Creutzfeldt-Jakob Diseases,Jacob Disease, Creutzfeldt,Jakob Creutzfeldt Disease,Jakob Creutzfeldt Syndrome,New Variant Creutzfeldt Jakob Disease,Spongiform Encephalopathies, Subacute,Subacute Spongiform Encephalopathies,Subacute Spongiform Encephalopathy,Syndrome, Creutzfeldt-Jakob,Syndrome, Jakob-Creutzfeldt,V CJD (Variant Creutzfeldt Jakob Disease),Variant Creutzfeldt Jakob Disease
D008822	Mice, Transgenic	Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.	Transgenic Mice,Founder Mice, Transgenic,Mouse, Founder, Transgenic,Mouse, Transgenic,Mice, Transgenic Founder,Transgenic Founder Mice,Transgenic Mouse
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
D001921	Brain	The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.	Encephalon
D002417	Cattle	Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.	Beef Cow,Bos grunniens,Bos indicus,Bos indicus Cattle,Bos taurus,Cow,Cow, Domestic,Dairy Cow,Holstein Cow,Indicine Cattle,Taurine Cattle,Taurus Cattle,Yak,Zebu,Beef Cows,Bos indicus Cattles,Cattle, Bos indicus,Cattle, Indicine,Cattle, Taurine,Cattle, Taurus,Cattles, Bos indicus,Cattles, Indicine,Cattles, Taurine,Cattles, Taurus,Cow, Beef,Cow, Dairy,Cow, Holstein,Cows,Dairy Cows,Domestic Cow,Domestic Cows,Indicine Cattles,Taurine Cattles,Taurus Cattles,Yaks,Zebus
D003670	Deer	The family Cervidae of 17 genera and 45 species occurring nearly throughout North America, South America, and Eurasia, on most associated continental islands, and in northern Africa. Wild populations of deer have been established through introduction by people in Cuba, New Guinea, Australia, New Zealand, and other places where the family does not naturally occur. They are slim, long-legged and best characterized by the presence of antlers. Their habitat is forests, swamps, brush country, deserts, and arctic tundra. They are usually good swimmers; some migrate seasonally. (Walker's Mammals of the World, 5th ed, p1362)	Deers
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
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
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