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Phenotypes Associated with This Genotype
Genotype
MGI:5491033
Allelic
Composition
Tg(Prnp*P101L)2866Sbp/0
Genetic
Background
involves: FVB/N
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phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die on average about 17 days following development of clinical signs of neurological disease

nervous system
• occasional prion protein plaques are seen in brains of a few ill mutants
• reactive astrocytic gliosis
• sciatic nerve shows regional loss of axons and active Wallerian degeneration
• sciatic nerve shows regional loss of axons
• mean age of neurologic disease onset is 220 +/- 10 days
• mean duration of illness (time from appearance of clinical signs to death) is 17 +/- 3 days
• sciatic nerve shows active Wallerian degeneration

muscle
• subsarcolemmal accumulation of mitochondria and abnormalities in distribution of the sarcoplasmic reticulum
• skeletal muscles of aged mutants exhibit neuropathic and myopathic changes around 200 days of age
• occasionally, degenerating skeletal muscle fibers are seen with rare fibers undergoing phagocytoisis
• skeletal muscle from clinically ill mutants shows type I fiber predominance and grouping indicative of neurogenic rearrangement of muscle fiber typesskeletal muscle from clinically ill mutants shows type I fiber predominance and grouping indicative of neurogenic rearrangement of muscle fiber types

homeostasis/metabolism
• sciatic nerve shows active Wallerian degeneration

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