Table 2.
Comparison of two hGH-iCJD (MM) cases with differing pathological and molecular features
| hGH-iCJD21 | hGH-iCJD20 | |
|---|---|---|
| PRNP codon 129 genotype | MM | MM |
| Neuropathology | Diffuse PrP positivity (resembling MM1 sCJD and hDM-iCJD) | PrP positive kuru plaques present (similar to MV2K sCJD) |
| RT-QuIC seeding activity | Shorter lag time (closer to MM1 sCJD) | Long lag time |
| PrPres | Type 1 (~21 kDa) | Type i (~20 kDa) |
| PMCA efficiency | Poor | Greater |
| PMCA substrate preference | None | VV > MM |
| PMCA product PrPres mobility | Type 1 | MM Substrate Results In Upshift VV Substrate Results In Downshift |