Abstract
Prion diseases are neurodegenerative and invariably fatal conditions that affect humans and animals. In particular, Creutzfeldt-Jakob disease (CJD) and bovine spongiform encephalopathy (BSE) are paradigmatic forms of human and animal prion diseases, respectively. Human exposure to BSE through contaminated food caused the appearance of the new variant form of CJD (vCJD). These diseases are caused by an abnormal prion protein named PrPSc (or prion), which accumulates in the brain and leads to the onset of the disease. Their definite diagnosis can be formulated only at post-mortem after biochemical and neuropathological identification of PrPSc. Thanks to the advent of an innovative technique named protein misfolding cyclic amplification (PMCA), traces of PrPSc, undetectable with the standard diagnostic techniques, were found in peripheral tissues of patients with vCJD, even at preclinical stages. The technology is currently being used in specialized laboratories and can be exploited for helping physicians in formulating an early and definite diagnosis of vCJD using peripheral tissues. However, this assay is currently unable to detect prions associated with the sporadic CJD (sCJD) forms, which are more frequent than vCJD. This review will focus on the most recent advances and applications of PMCA in the field of vCJD and other human prion disease diagnosis.
Highlights
Transmissible spongiform encephalopathies (TSEs) are a group of rare neurodegenerative conditions, known as prion diseases, that affect both humans and animals [1]
Cases of iatrogenic Creutzfeldt-Jakob disease (CJD) account for fewer than one percent of all known cases and have been reported in patients (1) subjected to corneal or dura mater grafts, (2) treated with human growth hormones or gonadotropins derived from the pituitary gland of cadavers, and (3) subjected to brain surgery with contaminated electrodes [10,11,12,13,14,15,16,17]
The variant form of Creutzfeldt-Jakob disease is a zoonotic disorder that appeared in humans after the consumption of food products from cattle affected by bovine spongiform encephalopathy (BSE) [21]
Summary
Transmissible spongiform encephalopathies (TSEs) are a group of rare neurodegenerative conditions, known as prion diseases, that affect both humans and animals [1]. The variant form of Creutzfeldt-Jakob disease (vCJD) is a zoonotic disorder that appeared in humans after the consumption of food products from cattle affected by bovine spongiform encephalopathy (BSE) [21]. BSE, known as mad cow disease, was first discovered in 1986 in the United Kingdom and reached an epidemic peak in 1992 [20] This resulted in stricter controls on cattle-derived food and many national CJD surveillance systems have been established [22,23,24,25,26,27]. Three of them developed the clinical signs of the disease and one remained asymptomatic [30] For this reason, prions might be present in many other carriers who may never develop vCJD, but are capable of transmitting the disease. No cases of sCJD transmission through blood transfusion have been described
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