Annotation Detail

Information
Associated Genes
PRNP
Associated Variants
PRNP p.Gln217Arg (p.Q217R) ( ENST00000379440.9, ENST00000424424.2, ENST00000430350.2, ENST00000457586.2 )
PRNP p.Gln217Arg (p.Q217R) ( ENST00000379440.9, ENST00000424424.2, ENST00000430350.2, ENST00000457586.2 )
Associated Disease
Gerstmann-Straussler-Scheinker Disease
Source Database
DisGeNET
Description
We have now studied the folding and turnover of PrP32 to understand the mechanism by which abnormal PrP forms cause cellular toxicity in our cell culture model and in the human brain carrying the Gerstmann-Sträussler-Scheinker disease Q217R mutation.
Pubmed
10970892
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.600051301892636
Year of publication
2000
Drugs