Spontaneous Disease in Mutant Transgenic Mice

Given that human 102L cases do transmit to wild-type mice (Tateishi et al, 1996), it is surprising that mouse PrPlOlL from spontaneously sick Tg(GSSPrP) 174 mice failed to transmit to wild-type mice but rather did transmit to hamsters where there is a natural mouse to hamster species barrier to overcome. 

Moreover, evidence that these diseases are transmissihle was not provided. 

It is conceivable that if these lOlL transgenic mice (Manson et al., 

1999) lived long enough to compensate for the low endogenous level of the mutant protein, they might develop spontaneous disease. Such a view is supported by tbe fact that humans with known pathogenic mutations do not develop disease until late in life. By the same token, overexpression of mutant PrP proteins may accelerate the process of spontaneous conversion, which will manifests as disease in the lifetime of the mice. 

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