All Phenotypes Prnp<tm2.1Cwe> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Prnp^tm2.1Cwe
targeted mutation 2.1, Charles Weissmann
MGI:2682338

Summary

3 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Prnp^tm2.1Cwe/Prnp^tm2.1Cwe	involves: 129P2/OlaHsd	MGI:2682349
hm2	Prnp^tm2.1Cwe/Prnp^tm2.1Cwe	Not Specified	MGI:3047649
ht3	Prnp^tm1Cwe/Prnp^tm2.1Cwe	involves: 129P2/OlaHsd	MGI:2682350

Allelic Composition	Prnp^tm2.1Cwe/Prnp^tm2.1Cwe
Genetic Background	involves: 129P2/OlaHsd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm2.1Cwe mutation (0 available); any Prnp mutation (148 available)

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

behavior/neurological

tremors ( J:67593 )

• mice began showing tremors and trembling gait at 6 months of age with incomplete penetrance

• observed in 50% of mice around 10 months of age

• observed in 100% of mice around 13 months of age

ataxia ( J:67593 )

muscle

abnormal muscle physiology ( J:67593 )

• progressive hypotonicity of hindlimbs

• more severe than in the forelimbs

nervous system

abnormal cerebellum morphology ( J:67593 )

decreased Purkinje cell number ( J:67593 )

• age-dependent loss of Purkinje cells

• ~40% loss in vermis I-VIII at 30 weeks

• ~80% loss in vermis I-VIII at 63 weeks

• normal granular layer, indicating Purkinje cell loss was not secondary to granular layer abnormalities

thin cerebellar molecular layer ( J:67593 )

• putatively due to the loss of the dendritic structures of Purkinje cells

Allelic Composition	Prnp^tm2.1Cwe/Prnp^tm2.1Cwe
Genetic Background	Not Specified

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm2.1Cwe mutation (0 available); any Prnp mutation (148 available)

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

nervous system

gliosis ( J:89232 )

• homozygous mutants develop gliosis

Allelic Composition	Prnp^tm1Cwe/Prnp^tm2.1Cwe
Genetic Background	involves: 129P2/OlaHsd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (43 available); any Prnp mutation (148 available)
Prnp^tm2.1Cwe mutation (0 available); any Prnp mutation (148 available)

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

behavior/neurological

tremors ( J:67593 )

• mice began showing ataxia and other cerebellar related phenotypes at 12 months of age

• observed in 50% of mice around 16 months of age

• observed in 100% of mice around 17 months of age

nervous system

abnormal cerebellum morphology ( J:67593 )

decreased Purkinje cell number ( J:67593 )

• age-dependent loss of Purkinje cells

• no significant loss at 30 weeks

• 30% loss at 63 weeks

• 70% loss at 95 weeks

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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