Phenotypes associated with this allele

• Allele Symbol: Tg(Prnp*S170N*N174T)1020Aag
• Allele Name: transgene insertion 1020, Adriano Aguzzi
• Allele ID: MGI:5569540
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp*S170N*N174T)1020Aag/0	involves: 129S7/SvEvBrd * C57BL/6	MGI:5569635

Genotype
MGI:5569635

cx1

• Allelic Composition: Prnp^tm1Cwe/Prnp^tm1Cwe; Tg(Prnp*S170N*N174T)1020Aag/0
• Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

Tg(Prnp*S170N*N174T)1020Aag/0 Prnp^tm1Cwe/Prnp^tm1Cwe mice develop a lethal neurologic disease with lesions in the brain and muscle

behavior/neurological

lethargy ( J:143528 )

ataxia ( J:143528 )

• circling movements, indicative of ataxia

paresis ( J:143528 )

circling ( J:143528 )

• circling movements

growth/size/body

weight loss ( J:143528 )

hematopoietic system

microgliosis ( J:143528 )

immune system

microgliosis ( J:143528 )

decreased susceptibility to prion infection ( J:143528 )

• mice inoculated with mouse-adapted Rocky Mountain Laboratory prions develop scrapie only after 323 +/- 92 days compared to Tg(Prnp)a20Cwe mice which develop scrapie after 74 +/- 6 days or wild-type mice that develop scrapie after 170 +/- 12 days

integument

increased pruritus ( J:143528 )

• occasionally pruritus is seen

muscle

abnormal muscle fiber morphology ( J:143528 )

• large, coarse PrP aggregates are seen in myofibers

muscle degeneration ( J:143528 )

• myofibers exhibit degeneration and regeneration, vary in size, and contain angular and split fibers and centralized nuclei

nervous system

microgliosis ( J:143528 )

abnormal brain morphology ( J:143528 )

• brains contain multicentric prion protein (PrP) deposits in the stratum lacunosum-moleculare of the hippocampus, within the corpus callosum, and in the cingulum

• PrP deposits are sometimes surrounded by vacuoles

• PrP deposits are often arranged in clusters of 25 to 50 particles and density of deposits varies

abnormal corpus callosum morphology ( J:143528 )

• multicentric prion protein deposits within the corpus callosum

abnormal cingulum morphology ( J:143528 )

• multicentric prion protein deposits in the cingulum

abnormal hippocampus stratum lacunosum morphology ( J:143528 )

• contain multicentric prion protein (PrP) deposits in the stratum lacunosum-moleculare

astrocytosis ( J:143528 )

abnormal sciatic nerve morphology ( J:143528 )

• sciatic nerves display 'onion bulbs' indicative of cycles of active demyelination and re-myelination, as well as macrophages and Schwann cells with myelin-filled vacuoles

spongiform encephalopathy ( J:143528 )

• 100% of mice develop spongiform encephalopathy; neurodegenerative onset occurs at 145 to 637 days of age, with 50% incidence by 364 days

skeleton

kyphosis ( J:143528 )