Abstract
In this review, the most important neuropathological changes found in the cerebella of sheep affected by classical natural scrapie are discussed. This disease is the oldest known of a group of unconventional “infections” caused by toxic prions of different origins. Scrapie is currently considered a “transmissible spongiform encephalopathy” (due to its neuropathological characteristics and its transmission), which is the paradigm of prion pathologies as well as many encephalopathies (prion-like) that present aberrant deposits of insoluble protein with neurotoxic effects due to errors in their catabolization (“misfolding protein diseases”). The study of this disease is, therefore, of great relevance. Our work data from the authors’ previous publications as well as other research in the field. The four most important types of neuropathological changes are neuron abnormalities and loss, neurogliosis, tissue vacuolization (spongiosis) and pathological or abnormal prion protein (PrP) deposits/deposition. These findings were analyzed and compared to other neuropathologies. Various aspects related to the presentation and progression of the disease, the involution of different neuronal types, the neuroglial responses and the appearance of abnormal PrP deposits are discussed. The most important points of controversy in scrapie neuropathology are presented.
Highlights
Classical scrapie is a naturally transmissible spongiform neurodegenerative disease that originally affected sheep, goats and mouflons and that has been observed for several centuries [1,2]
We aim to highlight the alterations studied in the cerebellum of sheep suffering from classical scrapie (Table 1) and their correlations observed in other mammals, including humans, to try to understand the neuropathology of all prion-like diseases
Loss and studied morphohistochemical alterations of Purkinje Cell layer neurons suffering from classical scrapie (Table 1) and their correlations observed in other mammals, including humans, to try to understand the neuropathology of all prion‐like diseases
Summary
Classical scrapie is a naturally transmissible spongiform neurodegenerative disease that originally affected sheep, goats and mouflons and that has been observed for several centuries [1,2]. The transfection of other animals has been performed in laboratory experiments designed to study the disease in greater depth [3,4,5,6] It is a “strange” disease that cannot be classified as a classical bacterial or viral infectious disease and does not follow the pathogenic patterns identified by infectious or neurodegenerative disease research. We collect and analyze the main data on cerebellum involution in classical scrapie of sheep to understand the neuropathology and clinical–pathological course of the disease in a unique natural model, which does not present the limitations observed in experimental research in the laboratory. Biomolecules 2021, 11, 649 the cerebellum will be analyzed to better understand these prion pathologies and identify possible therapeutic targets
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