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Phenotypes Associated with This Genotype
Genotype
MGI:5428333
Allelic
Composition
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp*)#Rgab/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnptm1Cwe mutation (32 available); any Prnp mutation (95 available)
Tg(Prnp*)#Rgab mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice are euthanized when they are too paralyzed to reach food and water

behavior/neurological
• in very few mice
• asymmetric hindlimb weakness at 5 to 6 months that progresses to paraplegia
• asymmetric hindlimb weakness at 5 to 6 months that progresses to paraplegia
• myoclonic jerks in some mice

limbs/digits/tail
• plastic tail in very few mice

muscle
• myoclonic jerks in some mice
• in the lower body

nervous system
• myoclonic jerks in some mice
• at 3 months and more prominent at 8 months
• at 3 months and more prominent at 8 months

skeleton

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Creutzfeldt-Jakob disease DOID:11949 OMIM:123400
J:183170


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
10/08/2019
MGI 6.14
The Jackson Laboratory