Advancing Prion Science Guidance for the National Prion Research Program (2004) / Chapter Skim
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Glossary
Pages 250-258
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From page 250... ...
It appears that some humans who ate beef or beef products containing the BSE agent have contracted variant Creutzfel~t-~akob disease, identified in 1996. Zoo animals fed beef or feed containing the BSE agent also have developed TSE.
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From page 251... ...
Transmitted among the three species by an unknown mechanism suspected to involve material shed from infected animals (e.g., urine, feces, saliva, hair, etch. There is no documented evidence that the disease is transmissible to humans, but it has been transmitted experimentally to cattle by intracerebral inoculation.
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From page 252... ...
haplotype: In general, refers to two sites, usually in proximity, along the same chromosome strand that are linked in terms of the phenotype they produce. In prion science, the term refers to the phenotypic effects of a mutation along the PRNP gene linked with a designated amino acid expressed by a polymorphic co(lon.
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From page 253... ...
Known cases of iC}D were caused by the transplantation of prior-contaminated human aura mater, injection of prior-contaminated human growth hormone from pituitary extracts, and reuse of surgical instruments and devices contaminated by prior use in a patient with C}D. ID50: The dose of an infectious agent sufficient to infect 50 percent of exposed animals or people.
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From page 254... ...
phenotype: With respect to PrP, relates to the metabolic, physiologic, and physical characteristics, both normal and pathological, exhibited by a host as a result of its PRNP gene expression interacting with the environment inside and outside the organism. polymorphism: Possession of two or more alleles of a gene that code for different amino acids at the same site along a protein sequence.
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From page 255... ...
However, a minority of respected TSE experts believe that the protein-only theory has not been proven beyond question. prion disease: A fatal, transmissible neurodegenerative disease associated with aggregates of PrPSC, an abnormally folded isoform of the cellular protein PrP encoded by the Prnp gene.
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From page 256... ...
With this knowledge, drug developers can target a specific binding site on the molecule to disrupt, enhance, or redirect its normal activity, thus interrupting the disease process. By contrast, traditional drug development typically begins with a range of potentially therapeutic chemical compounds that are narrowed down to the best candidates through empirical observation of their effects.
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From page 257... ...
Investigators conducting prion transmission studies use transgenes to convey unnatural molecular characteristics to experimental animals so as to circumvent the species barrier. For example, it is easier to transmit BSE to a transgenic mouse with a bovine transgene than to a normal mouse.
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From page 258... ...
Western blot: A technique used in molecular biology to detect and identify proteins in a test sample; also known as an immunoblot. A mixture of proteins is embedded in a slab of polyacrylamide gel and subjected to electrophoresis, during which an applied voltage causes the proteins to travel linearly toward the opposite end of the acrylamide slab at rates dependent on each protein's mass (measured in kilodaltons EkDa]
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