Abstract
Prions cause fatal and transmissible neurodegenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in small ruminants, and bovine spongiform encephalopathy (BSE). After the BSE epidemic, and the associated human infections, began in 1996 in the United Kingdom, general concerns have been raised about animal prions. We detected a prion disease in dromedary camels (Camelus dromedarius) in Algeria. Symptoms suggesting prion disease occurred in 3.1% of dromedaries brought for slaughter to the Ouargla abattoir in 2015–2016. We confirmed diagnosis by detecting pathognomonic neurodegeneration and disease-specific prion protein (PrPSc) in brain tissues from 3 symptomatic animals. Prion detection in lymphoid tissues is suggestive of the infectious nature of the disease. PrPSc biochemical characterization showed differences with BSE and scrapie. Our identification of this prion disease in a geographically widespread livestock species requires urgent enforcement of surveillance and assessment of the potential risks to human and animal health.
Highlights
Prions cause fatal and transmissible neurodegenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in small ruminants, and bovine spongiform encephalopathy (BSE)
Creutzfeldt-Jakob disease in humans and scrapie in small ruminants are the longest known diseases in this group, but prion diseases entered the public spotlight with the massive bovine spongiform encephalopathy (BSE) epidemic started in 1986 in the United Kingdom, revealing the zoonotic potential of animal prions
Public health concern increased markedly after variant Creutzfeldt-Jakob disease was demonstrated to be caused by the same prion strain responsible for the BSE epidemics [4]
Summary
Prions cause fatal and transmissible neurodegenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in small ruminants, and bovine spongiform encephalopathy (BSE). PrPSc biochemical characterization showed differences with BSE and scrapie Our identification of this prion disease in a geographically widespread livestock species requires urgent enforcement of surveillance and assessment of the potential risks to human and animal health. Creutzfeldt-Jakob disease in humans and scrapie in small ruminants are the longest known diseases in this group, but prion diseases entered the public spotlight with the massive bovine spongiform encephalopathy (BSE) epidemic started in 1986 in the United Kingdom, revealing the zoonotic potential of animal prions. Disorders resembling sporadic or genetic human prion diseases have been reported only recently, with the discovery of atypical/Nor scrapie in small ruminants [7] and L- and H-type BSE in cattle [8,9]. Even the outbreaks of transmissible mink encephalopathy reported in the United States and various European countries in ranch-raised mink most likely originated from feedstuff accidentally contaminated by prions [10]
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