Chronic wasting disease in transgenic mice and white-tailed deer - prion trafficking and pathogenesis
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Abstract
Chronic wasting disease (CWD) is a naturally occurring, horizontally transmitted prion disease of cervids, including deer, elk and moose. Typical of all prion diseases, CWD is characterized by the forced conversion of the normal prion protein (PrPC) into a misfolded isoform (PrPCWD), which accumulates in tissues of the lymphoid, endocrine, and nervous systems. The accumulation of PrPCWD in the nervous system results in a constellation of 4 microscopic brain lesions: (1) neuronal degeneration and/or loss, (2) neuronal and neuropil degenerative spongiosis, (3) reactive gliosis with absent-to-minimal ...
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