Urine sampling may help to detect Prion disease.

PrPSc, the only known component of the prion, is present mostly in the brains of animals and humans affected with prion diseases. The investigators shown that a protease resistant PrP isoform can also be detected in the urine of hamsters, cattle and humans suffering from TSEs. Most important, this PrP isoform (UPrPSc) was also found in the urine of hamsters inoculated with prions long before the appearance of clinical signs. Interestingly, i.c. inoculation of hamsters with UPrPSc did not cause clinical signs of prion disease even after 270 days, suggesting it differs in its pathogenic properties from brain PrPSc. The investigators propose that the detection of UPrPSc can be used to diagnose humans and animals incubating prion diseases.

Reference for further reading:
J Biol Chem 2001 Jun 21.A protease resistant PrP isoform is present in urine of animals and humans affected with prion diseases. Shaked GM, Shaked Y, Kariv Z, Halimi M, Avraham I, Gabizon R. Neurology, Hadassah University Hospital, Jerusalem 91120.