There are numerous TSE-related terms and abbreviations used within this site or within the literature; for simplicity most are defined or described here as used relative to material on this site:

Adapted - with respect to this web site adapted has an unique usage: when infection with PrPSc occurs across species the presentation of the disease changes, with subsequent passages within the same species the TSE assumes a stable presentation - at this point the TSE has 'adapted' to the new species

Amyloid - having the form or nature of starch, starch-like, some plaques found in TSE brains have this appearance but are the result of PrPSc 27-30 accumulation not starch

Antibody - protein produced by the body in response to an infection or artificially administered agent, antibodies bind and neutralize the infectuous agent, antibodies are often induced in animals by the injection of antigens, the antibodies found in the animals serum are used to develop assays to identify and quantify the antigen in biological samples (blood and tissues)

Antigen - usually a purified agent (protein) injected into an animal to elicit an immune response to form antibodies that recognize the antigen

Bovine - relating to cattle

BSE - bovine spongiform encephalopathy - form of TSE originally found in cattle, Mad Cow Disease

Caveolae - an intracellular compartment used to tranport molecules from the outside to the inside of the cell, has a neutral pH

Central nervous system - the brain and spinal cord

Cerebellum - a part of the brain

Cervids - members of the deer family

CJD - any of several human forms of TSE, Creutzfeldt-Jakob disease

CNS - see central nervous system

CtmPrP - a particular transmembrane (tm) form of the cellular (C) prion protein (PrP) that causes neurodegenerative changes similar to some genetic prion diseases; this disease is not covered in the web site. Although relating to prions, PrPSc (actually PrPRes) does not accumulate in the brain.

CWD - a form of TSE found in mule deer and elk, chronic wasting disease

ED50 - amount of material to infect 50% of those animals tested, one ED50 for PrPSc is ~100,000 molecules

Electrophoresis - see gel electrophoresis

Encephalopathy - a disease of the brain involving changes in brain structure

Endemic - constantly or regularly found

Exotic ungulate encephalopathy - a form of TSE found in greater kudus, nyalas, and oryx

Familial - inherited, i.e., passed on by genetic information in DNA from parent to progeny

Feline - relating to cats

FFI - a human form of TSE, fatal familial insomnia

Fidelity - degree of reproduction or transmission to which the new product resembles the original

Founder - the originator, with respect to TSE - the animal whereby the disease began

FSE - a form of TSE found in cats, felline spongiform encephalopathy

FSI - a human form of TSE, fatal sporadic insomnia

Gel electrophoresis - the separation of macromolecules (proteins) in an electrical field (usually separating by size)

Gene - section of DNA responsible for encoding a specific protein and containing additional elements for the regulation of its expression; when referring to the gene it is common to type the name in italics (Prnp) to avoid confusion with references to the protein encoded by the gene (PrP)

Gene knockout - this refers to the molecular biological removal of a gene, often used with mice as an experimental model to identify the function of a protein; i.e. examine mice with the gene and compare them to mice without a gene (Prnp+/+ = normal mouse with two copies of the mouse prion gene, Prnpo/o or Prnp-/- = mouse with no copies of the mouse prion gene, Prnp+/o or Prnp+/- = mouse with a single copy of the mouse prion gene often generated by breeding Prnp+/+ mice with either Prnpo/o or Prnp-/- mice)

Genetic Material - DNA and RNA, the chemical compounds that contain nucleid acids and the information on how and when to make all the various materials needed for life

Genotype - referring to the genetic makeup of an individual, each person contains two copies of each gene which may or my not be similar, different versions of a gene defines a new genotype

Glycosylation - the attachment of sugar residues to a protein, glycosylation stabilized protein structure and helps in localizing proteins to specfic sites on or within cells

GPI - a 'sticky foot' attached to proteins to anchor them to the cell surface; the 'sticky foot' contains sugar and lipid components (the lipid terminal portion is inserted into the lipid bilayer of the cell membrane - plasma membrane; proteins attached to cells by this mechanims can be removed by cleavage of the lipid portion with phosphatidyl-inositol specific-phospholipase C: PI-PLC)

Glycosylphosphatidyl-inositol-anchored glycoprotein - see GPI

GSS (D) - a group of several human forms of TSE, Gersmann-Straussler-Scheinker disease

Host - an animal or person that carries a disease from infection or transmission

Iatrogenic - caused unintentionally by a physician

Knockout - see gene knockout

Kuru - a human form of TSE

Offal - scraps or waste material, in this case the parts of carcasses that are inedible for humans

Mad Cow Disease - see BSE

MBM - meat and bone meal, a biproduct of animal slaughtering often used as a high protein feed

Meat and bone meal - see MBM

Murine - relating to mice

Neuropil (e) - nonmyelinated nerve fibers with numerous synapses (connections betweens neurons) in highly developed parts of the brain

nvCJD - see vCJD

Ovine - relating to sheep

Passage - within the scientific community this term has several usages, relative to TSE - passage is used to indicate that infectious material is collected from one animal and given to another; passage may be within species (ex. hamster brain extracts injected back into hamsters) or across species (ex. hamster brain extracts injected into mice)

Peripheral nervous system - the nervous system excluding the brain and spinal cord, the nerves delivering information from the sensory organs (ex. tongue, eyes, skin) to the brain and delivering signals from the brain to responding organs (ex. muscle, heart)

Phenotype - the sum of the genes which define of the observable features of an individual

Plaque - quadrangular, round, oval, or other regular form, usage here refers to abnormal shapes generally found in TSE brains

PNS - see peripheral nervous system

Presentation - with regards to medicine, presentation refers to all of the features associated with the disease (this includes clinical symptoms, period of time from infection till clinical features become evident, lesion location and size, etc.)

Prion - a protein of unknown function found in many tissues including the CNS

Protein-Only Theory - the scientific explanation for transmission of TSE diseases in which the only material needed to transmit the disease is the misfolded prion protein

Protein X - a protein that is believed to interact with PrPC and PrPSc within cells during the conversion of the former to the latter, acts as a molecular chaperone

Prn-i - see PRNP

PRNP or Prnp - the gene encoding the prion protein and all the regulatory elements thereof, the name may contain superscripts referring to various forms of the gene (i.e. PrnpC = the normal gene; PrnpE200K, 129M the gene associated with fCJD) or prefixes referring to the species of origin (i.e. HuPrnp = the human gene; SHaPrnp = the syrian hampster gene) or both if necissary (HuPrnpE200K, 129M)

PrP - see prion

PrP* - a proposed intermediate formed during the conversion of PrPC to PrPSc in conjunction with Protein X

PrP amyloid - aggregated fibrils usually composed of PrPSc 27-30 sometimes found in TSE brains

PrPBSE - the bovine (cattle) equivalent of PrPSc used to refer only to the bovine protein, largely replaced by boPrPSc or BoPrPSc

PrPC - the normal prion protein (c = cellular), this protein is not infectious, often prefixed to define a specific species when necessary (HuPrPC = human, BoPrPC = bovine, MoPrPC = mouse, OvPrPC = ovine/sheep, SHaPrPC = syrian hampster)

PrPC 27-30 - fragments of normal PrPC sometimes found in tissues (the 27-30 refers to the estimated size of the protein bands when analyzed by gel electroporesis; 27-30kDa or more correctly 27,000-30,000Mr)

PrPCJD - the human equivalent of PrPSc used to refer only to the human protein, largely replaced by huPrPSc or HuPrPSc

PrPM - refers to any mutant form of PrP such as the genetic mutations associated with CJD or GSSD, this term is used to avoid referring to the normal length protein found in patients with mutations as PrPC, specific identification of the mutation as a superscript may also be used when discussing the prion proteins found in several patients (ex. PrPC = normal full length prion protein, PrPE200K, 129M = full length prion protein found in fCJD, PrPD178N, M129 = full length prion protein found in FFI)

PrPRes - generally used to refer to PrPSc that is protease resistant (sometimes used to refer to any infectuous PrP causing TSE; but some PrPSc is not protease resistant, thus PrPSc is being used to refer to any infectuous PrP and PrPRes is being used only when referring to protease resistance per se)

PrP rod - polymer formed by the aggregation of PrPSc 27-30

PrPSc - the abnormal prion protein this protein is infectious and causes TSE (Sc = originally designated for Scrapie causing now used to refer to infectuous PrP for any form of TSE)

PrPSc 27-30 - PrPSc partilly digested with proteinase K, this form of PrPC is sometime protease resistant (the 27-30 refers to the estimated size of the protein bands when analyzed by gel electroporesis; 27-30kDa or more correctly 27,000-30,000Mr)

PrPSen - used to refer to PrP that is sensitive to proteinase K

Proteinase K - an enzyme that cleaves proteins, relatively unspecific, serine peptidase of the subtilism family clan SB, the enzyme used to classify proteolytic resistance of prion proteins

Render - to repeat or recite (rend - to pull apart by force or to produce my heating or melting); in the animal industry: the processing of offal to reuse for other purposes often animal feed or fertilizer usually by tearing/grinding apart and cooking

SAF - scrapie associated fibrils

secPrP - the mature form of PrP that has been translocated into the ER eventually to be attached to the cell membrane by a GPI whose cleavage leads to release (secretion : sec) of the protein

Serial passage - relative to TSE - passage is used to indicate that infectious material is collected from one animal and given to another; passage is always within the same species (ex. hamster hamster hamster hamster)

Sinc - see PRNP

Scrapie - the group of TSEs found in sheep

Somnolence - drowsiness, sleepiness

Species barrier - the inability of an infectuous agent from one species to infect individuals from another species

Spongiform - like a sponge, porous

Sporadic - occurring randomly without known cause

Strain - individuals sharing a common heritage or ancestry; in respect to TSEs - a single TSE sharing similar phenotypic expression or inheritance

Tg - see transgene

Transmissible - capable of being conveyed (transferred) from one animal to another; similar to infectious or contageous

Transgene - transgene, usually referring to the artificial insertion of a gene from one individual to another (often the gene is inserted into a single cell such as an egg which is used produce live animals); this may be done within or across species; ex., for research purposes mice were generated whereby MoPrnp was removed and HuPrnp added resulting in mice that lack the mouse gene (Prnpo/o) but have a human transgene (tgHuPrnp) resulting in mice that were Prnpo/o / tgHuPrnp

Transgenic - animals containing a transgene; i.e. transgenic mice with HuPrnp

TME - a form of TSE found in mink, transmissible mink encephalopathy

TSE - transmissible spongiform encephalopathy; the generic term used when referring to the numerous forms of prion-related diseases

Ungulate - hoof shaped, used to refer to any hoofed animal including cattle, horses, antelopes, gazelles, chamois, giraffes, etc.

Vacuolation - formation of vacuoles within tissues, i.e. small cavities or vesicles; in some clinical settings - the space left behind by the localized death of cells that is filled with body fluids or cellular debris, with regard to TSE the vacuoles are intracellular within neruons (in the neuropil) and are typically round to oval (5-25µm in diameter, i.e. 0.0002-0.001 inches in diameter) but may be larger (~50µm in diameter)

vCJD - a human TSE (CJD) believed to be caused by the transmissible agent responsible for BSE

Virino - a small virus-like organism, proposed as the organism containing gentetic material involved in the transmission and fidelity of TSE diseases