2020
DOI: 10.1186/s40478-020-00958-x
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Transmission characteristics of heterozygous cases of Creutzfeldt-Jakob disease with variable abnormal prion protein allotypes

Abstract: In the human prion disease Creutzfeldt-Jakob disease (CJD), different CJD neuropathological subtypes are defined by the presence in normal prion protein (PrP C) of a methionine or valine at residue 129, by the molecular mass of the infectious prion protein PrP Sc , by the pattern of PrP Sc deposition, and by the distribution of spongiform change in the brain. Heterozygous cases of CJD potentially add another layer of complexity to defining CJD subtypes since PrP Sc can have either a methionine (PrP Sc-M129) or… Show more

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Cited by 6 publications
(6 citation statements)
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References 42 publications
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“…Processing of brains from RML and NBH inoculated mice for the detection of PrP Sc as well as SDS-PAGE, transfer of proteins to polyvinylidene difluoride (PVDF) membrane (Millipore, Burlington MA), and membrane development were all done as described previously [ 47 ]. For detection of proteins other than PrP Sc , digestion with proteinase K was omitted.…”
Section: Methodsmentioning
confidence: 99%
“…Processing of brains from RML and NBH inoculated mice for the detection of PrP Sc as well as SDS-PAGE, transfer of proteins to polyvinylidene difluoride (PVDF) membrane (Millipore, Burlington MA), and membrane development were all done as described previously [ 47 ]. For detection of proteins other than PrP Sc , digestion with proteinase K was omitted.…”
Section: Methodsmentioning
confidence: 99%
“…We then tested sfRT-QuIC in a more clinically relevant scenario. We used surgical instruments (forceps and scissors) that had been used to cut brain tissue from sCJD-infected transgenic mice expressing only human PrP (Tg66) [ 46 ], dry wiped with gauze (n = 2) or not (n = 3), and then air-dried prior to the application of SM ( Fig 2C ). For three of the instruments, including a wiped scissor, SM dilutions of 10 −2 –10 −5 were positive in all quadruplicate wells, while SMs from the other two instruments were uniformly positive out to 10 −3 and 10 −4 dilutions ( Fig 7 ).…”
Section: Resultsmentioning
confidence: 99%
“…The incubation periods of different type 1 and 2 brain homogenates in humanized mice vary in the literature [ 23 , 33 35 ]. This is at least partially due to the mouse strain, which may have different PrP C expression levels, and classification system used to define the subtypes.…”
Section: Discussionmentioning
confidence: 99%
“…Tg66 transgenic mice overexpressing human PrP C were used for this study. These mice have been shown to be susceptible to prion disease by infection with MV1 and 2 sCJD prions, with distinct disease courses and biochemistry apparent with the different inocula [ 23 ]. Generation of these mice was described previously [ 22 ].…”
Section: Methodsmentioning
confidence: 99%