Biomaterial Database

Amyloidoses of the nervous system in the transmissible dementias. 1992

P Piccardo

Department of Pharmacology and Therapeutics, McGill University, Montreal, Quebec, Canada.

The transmissible spongiform encephalopathies, both in humans and in animals, are neurodegenerative diseases which do not evoke an immune response in the host. The search for the etiological agent has led to the prion hypothesis, which proposes that a host-encoded protein may be the causal agent itself or a part of it. In humans, a low percentage of these transmissible encephalopathies are familial. Investigations centered on the understanding of the pathogenesis of the transmissible spongiform encephalopathies have implications, not only in basic sciences, but in clinical medicine as well.

UI	MeSH Term	Description	Entries
D008322	Mammals	Warm-blooded vertebrate animals belonging to the class Mammalia, including all that possess hair and suckle their young.	Mammalia,Mammal
D009154	Mutation	Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.	Mutations
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
D006224	Cricetinae	A subfamily in the family MURIDAE, comprising the hamsters. Four of the more common genera are Cricetus, CRICETULUS; MESOCRICETUS; and PHODOPUS.	Cricetus,Hamsters,Hamster
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000686	Amyloidosis	A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. Various signs and symptoms depend on the location and size of the deposits.	Amyloidoses
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
D013045	Species Specificity	The restriction of a characteristic behavior, anatomical structure or physical system, such as immune response; metabolic response, or gene or gene variant to the members of one species. It refers to that property which differentiates one species from another but it is also used for phylogenetic levels higher or lower than the species.	Species Specificities,Specificities, Species,Specificity, Species
D017096	Prion Diseases	A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83)	Dementias, Transmissible,Spongiform Encephalopathies, Transmissible,Transmissible Dementias,Encephalopathies, Spongiform, Transmissible,Human Transmissible Spongiform Encephalopathies, Inherited,Inherited Human Transmissible Spongiform Encephalopathies,Prion Disease,Prion Protein Diseases,Prion-Associated Disorders,Prion-Induced Disorder,Prion-Induced Disorders,Transmissible Spongiform Encephalopathies,Dementia, Transmissible,Disorder, Prion-Induced,Disorders, Prion-Induced,Encephalopathies, Transmissible Spongiform,Encephalopathy, Transmissible Spongiform,Prion Induced Disorder,Prion Protein Disease,Spongiform Encephalopathy, Transmissible,Transmissible Dementia,Transmissible Spongiform Encephalopathy
D051379	Mice	The common name for the genus Mus.	Mice, House,Mus,Mus musculus,Mice, Laboratory,Mouse,Mouse, House,Mouse, Laboratory,Mouse, Swiss,Mus domesticus,Mus musculus domesticus,Swiss Mice,House Mice,House Mouse,Laboratory Mice,Laboratory Mouse,Mice, Swiss,Swiss Mouse,domesticus, Mus musculus