Fibrils from brains of cows with new cattle disease contain scrapie-associated protein

Abstract

During the past two years, more than 1,000 cases of a neurological disorder of cattle, bovine spongiform encephalopathy (BSE), have been confirmed from farms throughout Great Britain. The neurological signs and brain pathology of BSE resemble those produced in other species by the pathogens of scrapie and related disorders. The discovery of fibrils similar to scrapie-associated fibrils in detergent extracts o BSE-affected brain supported the clinical and pathological diagnosis of the disease, but has been controversial. Scrapie-associated fibrils are found in brain extracts of all species affected by scrapie and diseases caused by related pathogens. They are pathological aggregates of a neuronal membrane protein termed PrP and a protease-resistant form of PrP is a molecular marker of scrapie-associated fibrils. In this report, we show the major protein of BSE fibrils is the bovine homologue of PrP as judged by its size, protease resistance, immunoreactivity, lectin binding and partial N-terminal protein sequence. This confirms that BSE is a scrapie-like disease.

Canine MDCK cell lines are refractory to infection with human and mouse prions

“…BSE has been transmitted to humans through the consumption of contaminated beef, resulting in the emergence of a new human prion disease, named variant CJD (vCJD), affecting predominantly young adults [31]. Following the occurrence of BSE in three continents since 1986 [32][33][34] the need to ensure the TSE safety of vaccines has become vital. One approach is to avoid, whenever possible, tissues from animals, risk tissues and tissues of unknown or uncertain provenance [35].…”

The yeast prion protein Ure2: Structure, function and folding

“…The prion diseases include CreutzfeldtJacob disease (CJD) in humans, and bovine spongiform encephalopathy (BSE) and scrapie in animals. The BSE epidemic in the UK [2], and subsequent appearance of a new variant of the human disease [3], has highlighted the importance of research in this area. However, despite more than a decade of intensive research, the molecular mechanism of prion propagation, and even the normal function of the mammalian prion protein, PrP, remain mysterious.…”

Iron, Aging, and Neurodegeneration

“…They are more widely known because of the concurrent outbreaks of both bovine spongiform encephalopathy (BSE) in cattle and variant Creutzfeldt-Jakob disease (vCJD) in humans [116,117]. The potential transmissibility of prion diseases remains an ever-present concern.…”