In 1996, a new variant of Creutzfeldt-Jakob disease (vCJD)-a disease that causes lack of coordination, muscle twitching or jerking, dementia, and, eventually, death-suddenly appeared in Great Britain. It is believed that the victims contracted the disease from eating the beef of cattle stricken with bovine spongiform encephalopathy (BSE), or mad cow disease. As of December 1997, at least 25 peo...

A long-running scare story Europe has seen its share of food crises in recent times. Some are fairly short-lived, such as the scandal over wines contaminated with antifreeze and vegetables con taining unacceptably high levels of pesticide residues. Others last longer, as was the case with so-called “mad cow” disease – bovine spongiform encephalopathy (BSE) – caused by feeding meal made from pro...

BACKGROUND Transmissible Spongiform Encephalopathies (TSEs), including scrapie in sheep, chronic wasting disease (CWD) in cervids, transmissible mink encephalopathy (TME), and bovine spongiform encephalopathy (BSE), are fatal diseases of the nervous system associated with accumulation of misfolded prion protein (PrP(Sc)). Different strains of TSEs exist, associated with different PrP(Sc) confor...

The authors present a basic quantitative spreadsheet model to evaluate the risk of bovine spongiform encephalopathy (BSE) within a national setting. The model is based on information from BSE risk assessments undertaken in Latin American countries. The analysis focuses on the level of regulatory implementation and its impact over different time periods and estimates the potential impact if one ...

The risks posed to human health by individual animal prion diseases cannot be determined a priori and are difficult to address empirically. The fundamental event in prion disease pathogenesis is thought to be the seeded conversion of normal prion protein to its pathologic isoform. We used a rapid molecular conversion assay (protein misfolding cyclic amplification) to test whether brain homogena...

New variant Creutzfeldt-Jakob disease (nvCJD) is a novel human transmissible spongiform encephalopathy which was first identified in 1996 in the United Kingdom (UK). Subsequent scientific studies have revealed that the strain of the transmissible agent responsible for nvCJD is identical to that of the bovine spongiform encephalopathy (BSE) agent, and the disease has been considered as 'human BS...

© 2000 Canadian Medical Association or its licensors In the mid-1980s bovine spongiform encephalopathy (BSE) was recognized as an emerging prion disease of epidemic proportions in the United Kingdom. In April 1996, researchers in the UK announced that they had identified 11 patients with a new form of spongiform encephalopathy, now called variant Creutzfeldt–Jakob disease (vCJD). Since then, mo...

Transmissible spongiform encephalopathies are neurodegenerative diseases for which prions are the attributed pathogenic agents. A widely accepted theory assumes that prion replication is due to a direct interaction between the pathologic (PrP(Sc)) form and the host-encoded (PrP(C)) conformation, in a kind of autocatalytic process. Here we show that the overall features of the incubation time of...

In this work we show evidence of mother-to-offspring transmission in a transgenic mouse line expressing bovine PrP (boTg) experimentally infected by intracerebral administration of bovine spongiform encephalopathy (BSE) prions. PrP(res) was detected in brains of newborns from infected mothers only when mating was allowed near to the clinical stage of disease, when brain PrP(res) deposition coul...