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ACDP: UK government Advisory Committee on Dangerous Pathogens which regularly produces information regarding the handling of dangerous microorganisms.
ADAS: Agricultural Development and Advisory Service. UK government organisation that providing veterinary and agricultural advice to farmers. Greatly reduced in size during the Thatcher Government.
Agent: An element able to transmit a disease.
Alzheimer's disease: The most common dementing illness of the elderly in industrialized countries.
Amyloid: A chemical structure which can accumulate in brain or other tissues in an amorphous way as a result of a variety of different diseases. In CJD the amyloid is made-up of the prion protein.
Amyloidosis: The build up of amyloid which causes damage to the tissues in the body.
Astrocyte: Common cell type in the brain that supports and assists the function of the nervous tissue (neurons). Sometimes called an 'accessory cell'.
Astrocytosis: The proliferation of astrocytes in brain tissue due to the death of neurons (nerve cells). A common finding in CJD but it not specific to the disease.
Ataxia: Loss of muscular coordination resulting in jerky, uncontrolled movement.
BSE: Bovine spongiform encephalopathy (mad cow disease, la vache folle, etc).
Chronic wasting disease: TSE of deer and elk.
CJD: Creutzfeldt-Jakob disease. The most common human spongiform encephalopathy (SE). Identified in the 1920s through the work of Creutzfeldt and Jakob.
CNS: Central nervous system, i.e. the brain and the spinal cord.
CSF: Cerebrospinal fluid. A clear fluid that surrounds the brain and spinal cord.
CVL: UK government Central Veterinary Laboratory, which performed much of the early work on BSE.
DNA: Deoxyribonuclic acid. The chemical that carries the genes in the chromosomes of animals, plants and some viruses.
DoH: The UK Department of Health.
Downer cows: A term used in the US to describe cattle that do not have the ability to stand.
EEG: Electroencephalogram. The trace of electrical activity in the brain. In patients with CJD this may show characteristic signs.
Epidemiology: Study of the distribution of cases of a disease.
EU: European Union.
FFI: Fatal Familial Insomnia. A genetic disease in humans caused by an inherited mutation of the prion protein gene.
FSE: Feline spongiform encephalopathy. SE in domestic cats, believed to be related to the consumption of BSE material in cat food.
Gene: Unit of DNA and forming part of chromosome which is responsible for particular functions within a cell function.
Genome: The total make-up of all the genes of an organism.
Genotype: The genetic make-up of an individual.
GSS: Gerstmann-Straussler-Scheinker Disease. A familial SE of humans and associated with inherited mutations of the PrP gene.
Horizontal transmission: Lateral transmission of a disease from one animal to another (c.f. vertical transmission).
Iatrogenic: A disorder caused by medical treatment, e.g. CJD cases resulting from injections of growth hormone or transplants of tissue from an infected donor.
Immunohistochemistry: Chemical methods used to locate deposits of particular chemicals or proteins in tissues. Often used to identify the prion protein in brain tissue.
Kuru: TSE found only in the Fore tribe in New Guinea, related to ritualistic cannibalism carried out among members of the tribe.
MAFF: UK Ministry of Agriculture Fisheries and Food.
Maternal transmission: The transmission of disease from the mother to the offspring.
MBM: Meat and bone meal.
MRM: Mechanically recovered meat, stripped from the carcasses of animals & used in processed foods, e.g. hamburgers.
Mutation: A chemical change in a gene of a cell which alters or destroys its normal function.
Myoclonus: Involuntary muscular contractions.
Neuroanatomy: Anatomy of the brain, spinal cord and peripheral nervous system.
Passage: The inoculation of an infectious agent into an animal.
Pathogenesis: The production of damage (pathology) in a tissue or animal.
Pathology: Study of disease-affected tissues.
Plaques: Accumulation of protein that may build up in brain tissue as a result of infection.
Prion: PROteinaceous INfectious agent. Term coined by Stanley Prusiner in 198?. The prion theory suggests that the infectious agent of CJD and the other TSEs is composed only of a single protein and does not contain a nucleic acid genome which would be necessary if the agent was a conventional virus.
PrP: Prion protein. A normally occurring protein found on the surface of particular cell types - PrPC. The abnormal form PrPCJD (or PrPSc in scrapie) accumulates in the brain.
SAF: Scrapie associated fibrils. Filamentous structures, visible only in an electron microscope found only in brains from TSE.
SBO: Specified bovine offals. Offal of cows thought to represent a risk of BSE transmission. A ban on the use of these organs (thymus, brain, spinal cord, gut (below duodenum), spleen, tonsils) in the food chain was introduced (but not properly enforced) in 1989.
SBM: Specified bovine materials. Additional parts of slaughtered cows considered not acceptable for human consumption from December 1995.
Scrapie: TSE of sheep or goats.
SE: Spongiform encephalopathy.
SEAC: Spongiform Encephalopathy Advisory Committee. UK government committee consisting of 'experts' in the field of spongiform encephalopathy.
Species barrier: A naturally occurring barrier between different species of animal which makes transfer of TSE from one to the other difficult.
Sporadic: Cases of CJD that occur at random throughout the world are said to be sporadic if they are not associated with a genetic mutation or any known iatrogenic cause.
Strains: Different forms of the scrapie have been isolated by transmission to mice. These strains show distinct variations in the incubation period and pattern of pathology observed in the infected mice.
Thirty Month Rule: Regulation introduced in April 1996 that cattle over 30 months of age should not be used for human food.
Transgenic mice: Mice carrying a specific gene from another animal. There may be more than one copy of the gene (for instance, in many transgenic mice containing the human PrP gene there may be over 20 copies) and may actually replace the gene that is there normally. The gene is unlikely to be at the same site as the normal gene in the animal chromosome. To insert the gene requires the DNA to be inserted into the fertilised ovum of the animal and hence is very difficult indeed, with many failures.
TME: Transmissible mink encephalopathy. TSE of mink found on mink farms in the USA, first reported in the 1960s.
TSE: Transmissible spongiform encephalopathy. A disease that can be transmitted from one animal to another which causes microscopic holes in the brain that similar to a sponge.
Vertical transmission: Transmission of an illness from a parent to its offspring.
Virino: Alternative hypothesis to explain TSEs. Proposed to contain protein and nucleic acid, but distinct from conventional viruses.
Virus: An extremely small infectious agent with a specific structure and nucleic acid genome, able to cause its own multiplication after infection of cells.