However, the VPSPr prion shares the multiplicity of the resPr[P.sup.D] electrophoretic bands with prions from a subset of inherited prion diseases referred to as Gerstmann-Straussler-Scheinker disease
(GSS), prompting the suggestion that VPSPr is the sporadic form of GSS (7,10).
The genetic forms of prion disease are fatal familial insomnia and Gerstmann-Straussler-Scheinker disease
[4, 9, 11, 12].
In humans, they comprise Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (FFI), kuru, Gerstmann-Straussler-Scheinker disease
(GSS), and the recently described variably protease-sensitive prionopathy (VPSPr), whereas natural TSEs in animals include scrapie of sheep and goats, bovine spongiform encephalopathy (BSE), and chronic wasting disease (CWD) in deer and elk.
Tateishi, "The presence of complements in amyloid plaques of creutzfeldt-Jakob disease and gerstmann-straussler-scheinker disease
," Applied Pathology, vol.
These include Classic Creutzfeldt-Jakob disease (CJD), which occurs at a rate of one in a million people/year, Gerstmann-Straussler-Scheinker disease
(GSS) at a rate of five in 100 million people/year, and fatal familial insomnia, which has been characterized in 50 families [11,14, 15].
Less common are the transmissible spongiform encephalopathies (TSEs), including Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease
(GSSD), and fatal familial insomnia (FFI).
The human diseases include Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease
, and fatal familial insomnia.
showing protein amyloid deposits in the peripheral regions of PrP immunoreactive amyloid plaques.
Except for 1 patient with Gerstmann-Straussler-Scheinker disease
, all of these patients had sCJD.