prion diseases


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Related to prion diseases: Gerstmann-Straussler-Scheinker syndrome

prion diseases

[′Prī‚än di‚zēz·əz]
(medicine)
A group of invariably fatal disorders affecting humans and animals that are clinically characterized by neurological and behavioral degeneration caused by the cerebral accumulation of an abnormal prion protein which is resistant to proteolytic enzymes and, in contrast to other infectious agents, does not require nucleic acid for replication. The diseases are transmissible either genetically (for example, Creutzfeldt-Jakob disease) or via infection (new variant Creutzfeldt-Jakob disease and mad cow disease) or can occur spontaneously (classical or sporadic Creutzfeldt-Jakob disease). Also known as spongiform encephalopathies.
References in periodicals archive ?
Mice with only the protective variant didn't get prion diseases, including vCJD, when injected with prions.
Prion diseases have the potential to be transmissible between persons, a fact that raises public health concerns, particularly regarding vCJD.
Further, a test that identifies people with various forms of prion diseases could help to prevent their spread among and between species.
The histological pathology found in animals affected by CWD is consistent with that of other animal prion diseases.
Brian Appleby, a neuropsychiatrist at the Cleveland Clinic Lou Ruvo Center for Brain Health, speculated that a blood test could have "huge implications" for screening the blood supply, largely in the United Kingdom, and for use in checking deer and elk for chronic wasting disease, the main acquired prion disease seen in the United States.
That was important because any drug candidate for prion diseases would have to be able to cross the blood-brain barrier to reach the brain.
A cohort study of the impact of prion disease on farm family community health (Rural Family Health) is currently underway, led by Wilfreda E.
Prion diseases can be inherited, as in fatal familial insomnia (the Italian family that couldn't sleep): they include other fatal neurological disorders such as Mad Cow and scrapies.
Max, who himself has a neuromuscular disorder loosely related to the diseases he describes, wants to make a few political points about the relationship between prion diseases and government agricultural policies.
Healthy priori protein-free calves should be useful in investigating the function of normal cellular prion protein and the nature of prion diseases.
The prion diseases are characterized by the post-translational modification of a naturally occurring neuronal surface protein--Prion Protein Cellular (PrPc).
Prion diseases are caused when abnormal prion proteins in the animal's body change the form of normal prion proteins.