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Phenotypes Associated with This Genotype
Genotype
MGI:5491048
Allelic
Composition
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp*P101L)2866Sbp/0
Genetic
Background
involves: 129S7/SvEvBrd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnptm1Cwe mutation (29 available); any Prnp mutation (88 available)
Tg(Prnp*P101L)2866Sbp mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mutants die within about 3 days following clinical signs of neurological disease

nervous system
• brains of ill mutants contain abundant prion protein amyloid plaques; prion protein deposits are not resistant to proteinase K digestion
• mean age for development of neurologic disease is 146 +/- 2 days
• mean duration of illness (time from appearance of clinical signs to death) is reduced from 17 +/- 3 days in single Tg(Prnp*P101L)2866Sbp mice to 3 +/-1 days in double mutants

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Gerstmann-Straussler-Scheinker syndrome DOID:4249 OMIM:137440
J:136434


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/12/2019
MGI 6.13
The Jackson Laboratory