Table I.

Characteristics of transgenic mice

Transgenea Expression levelb CtmPrP in vitroc CtmPrP in cells CtmPrP in vivod Time to disease 
  % %  d 
PrP(A3922) 4.0× ∼10 <2 <2 >700 
PrP(KH-II)H 3.0× ∼30 ∼10 ∼10–20 ∼60 
PrP(KH-II)M 0.6× ∼30 ∼10 ∼5–8 ∼470 
PrP(A117V)H 4.0× ∼15 ∼4 ∼5–8 ∼570 
PrP(AV3) 1–4× ∼35 ∼10 ∼10–20 <60 
Prl-PrP(AV3)6 4.7× ∼15 <2 2–5 >600 
Prl-PrP(AV3)10 2.4× ∼15 <2 <2 >600 
Prl-PrP(AV3)11 5.7× ∼15 <2 ∼5–8 ∼200 
HuPrP(A117V)36 2.4× ∼15 ∼4 NDe ∼560 
Opn-(A117V)33 4.0× ∼10 <2 NDe >700 
a

The first five transgenic lines have been described previously (Hegde et al., 1998a, 1999).

b

Relative to PrP levels in normal hamster (defined as 1×).

c

Percentage of total PrP from a typical experiment. Exact amounts vary depending on experimental conditions and variations in the translation extract and ER microsomes. However, the relative relationships remain constant.

d

Arbitrary units; determined by comparing the amount of the diagnostic 18-kD band generated by limited PK digestion to a serial dilution of total brain homogenate.

e

Indicates not determined; the precise amounts could not be quantified because human PrP seems to behave slightly differently than rodent PrP in the limited PK digestion assay.

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