All Phenotypes Prnp<tm1Cwe> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Prnp^tm1Cwe
targeted mutation 1, Charles Weissmann
MGI:1888773

Summary

29 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Prnp^tm1Cwe/Prnp^tm1Cwe	involves: 129S7/SvEvBrd * C57BL/6	MGI:2174709
ht2	Prnp^tm1Cwe/Prnp^tm2.1Cwe	involves: 129P2/OlaHsd	MGI:2682350
cn3	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Nefh-cre)22Jcol/? Tg(Prnp)46Jcol/?	involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * FVB	MGI:3761844
cn4	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Nefh-cre)22Jcol/? Tg(Prnp)37Jcol/?	involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * FVB	MGI:3761924
cn5	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Nefh-cre)22Jcol/Tg(Nefh-cre)22Jcol Tg(Prnp)46Jcol/Tg(Prnp)46Jcol	involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * FVB	MGI:3761845
cx6	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PRNP)45Jcol/Tg(PRNP)45Jcol	involves: 129 * 129S7/SvEvBrd * C57BL/6 * FVB/N	MGI:5760247
cx7	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PRNP)35Jcol/Tg(PRNP)35Jcol	involves: 129 * 129S7/SvEvBrd * C57BL/6 * FVB/N	MGI:5760246
cx8	Prnp^tm1Cwe/Prnp^tm1Cwe Sprn^tm1Geno/Sprn^tm1Geno	involves: 129S2/SvPas * 129S7/SvEvBrd * C57BL/6J * FVB/N	MGI:5428672
cx9	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PrnpA116VM128V)1309Jama/0	involves: 129S7/SvEvBrd	MGI:4356193
cx10	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PrnpS170NN174T)1020Aag/0	involves: 129S7/SvEvBrd * C57BL/6	MGI:5569635
cx11	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Lck-Prnp)33Cwe/0	involves: 129S7/SvEvBrd * C57BL/6	MGI:4821407
cx12	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)C4Cwe/0	involves: 129S7/SvEvBrd * C57BL/6	MGI:4821387
cx13	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)a20Cwe/0	involves: 129S7/SvEvBrd * C57BL/6	MGI:4821386
cx14	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PrnpD177NM128V)A21Rchi/Tg(PrnpD177NM128V)A21Rchi	involves: 129S7/SvEvBrd * C57BL/6 * CBA	MGI:3836994
cx15	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PrnpD177NM128V)A21Rchi/0	involves: 129S7/SvEvBrd * C57BL/6 * CBA	MGI:3836995
cx16	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp-tTA)F959Sbp/0 Tg(tetO-ATXN3)2904Olri/0	involves: 129S7/SvEvBrd * C57BL/6 * FVB	MGI:4410602
cx17	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp-tTA)F959Sbp/Tg(Prnp-tTA)F959Sbp Tg(tetO-ATXN3)2904Olri/0	involves: 129S7/SvEvBrd * C57BL/6 * FVB	MGI:4410603
cx18	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp-tTA)F959Sbp/0 Tg(tetO-ATXN3)2904Olri/Tg(tetO-ATXN3)2904Olri	involves: 129S7/SvEvBrd * C57BL/6 * FVB	MGI:4410604
cx19	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp-tTA)F959Sbp/Tg(Prnp-tTA)F959Sbp Tg(tetO-ATXN3)2904Olri/Tg(tetO-ATXN3)2904Olri	involves: 129S7/SvEvBrd * C57BL/6 * FVB	MGI:4410605
cx20	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp*)#Rgab/0	involves: 129S7/SvEvBrd * C57BL/6 * FVB/N	MGI:5428333
cx21	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PRNP)23454Sbp/0	involves: 129S7/SvEvBrd * FVB/N	MGI:6279179
cx22	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)4053Sbp/0	involves: 129S7/SvEvBrd * FVB/N	MGI:5491047
cx23	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp*P101L)2866Sbp/0	involves: 129S7/SvEvBrd * FVB/N	MGI:5491048
cx24	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PrnpP101L)2866Sbp/Tg(PrnpP101L)2866Sbp	involves: 129S7/SvEvBrd * FVB/N	MGI:5491050
cx25	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)37Jcol/?	involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * FVB	MGI:3761842
cx26	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)46Jcol/?	involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * FVB	MGI:3761843
cx27	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)c35Cwe/0	Not Specified	MGI:3531093
cx28	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)a19Cwe/0	Not Specified	MGI:3531091
cx29	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)a20Cwe/0	Not Specified	MGI:3531092

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)

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

behavior/neurological

behavior/neurological phenotype ( J:472 , J:67593 )

N	• normal behavior (J:472) • no ataxia or Purkinje cell loss observed (J:67593)

nervous system

decreased brain copper level ( J:126728 )

• after 12 months, copper levels in the brain fail to increase with age unlike in wild-type mice

• copper levels in the synaptosomes are decreased compared to in wild-type mice

abnormal CNS synaptic transmission ( J:19448 )

• GABA(A) receptor-mediated fast inhibition is weaker in hippocampal slices of mutants than in wild-type mice, indicating impaired synaptic inhibition

abnormal inhibitory postsynaptic currents ( J:19448 )

• inhibitory postsynaptic currents (ipscs) have significantly slower rising phases and are slower

abnormal inhibitory postsynaptic potential ( J:19448 )

• fast inhibitory postsynaptic potentials (ipsps) appear weaker in hippocampal slices than in wild-type, however excitatory postsynaptic potentials are normal

reduced long-term potentiation ( J:19448 )

• long term potentiation in CA1 of hippocampal slices is weaker in both the absence and presence of bicuculline

homeostasis/metabolism

decreased brain copper level ( J:126728 )

• after 12 months, copper levels in the brain fail to increase with age unlike in wild-type mice

• copper levels in the synaptosomes are decreased compared to in wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
NOT	Creutzfeldt-Jakob disease	DOID:11949	OMIM:123400	J:472
NOT	Gerstmann-Straussler-Scheinker syndrome	DOID:4249	OMIM:137440	J:472

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 (39 available); any Prnp mutation (150 available)
Prnp^tm2.1Cwe mutation (0 available); any Prnp mutation (150 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

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Nefh-cre)22Jcol/? Tg(Prnp)46Jcol/?
Genetic Background	involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * FVB

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Nefh-cre)22Jcol mutation (0 available)
Tg(Prnp)46Jcol mutation (0 available)

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

nervous system

abnormal afterhyperpolarization ( J:74652 )

• both slow and medium after hyperpolarization (AHP) responses are decreased compared to in wild-type mice (slow AHP, -0.97+/-0.13 mV compared to -0.3+/-0.05 mV in wild-type mice; medium AHP, -1.8+/-0.15 mV to -1.35+/-0.1 mV in wild-type mice)

• however, resting potentials, input resistances, and action potential thresholds and amplitudes are normal

immune system

decreased susceptibility to prion infection ( J:74652 )

• no mice develop scrapie for up to 400 days post-infection with no evidence of scrapie or accumulation of Prnp^Sc in one mouse that was culled due to intercurrent illness

behavior/neurological

behavior/neurological phenotype ( J:74652 )

N	• mice are indistinguishable from wild-type mice with normal gait, posture, motor control, coordination, autonomic function, general excitability and aggression at time points when cre is expressed, 3 to 5 months and 6 to 15 months

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Nefh-cre)22Jcol/? Tg(Prnp)37Jcol/?
Genetic Background	involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * FVB

Find Mice

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

behavior/neurological

abnormal behavior ( J:126424 )

• mice display behavioral defects despite normal grip strength and coordination of movement

• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display reduced burrowing (displacing 54+/-7% of pellets compared to 73+/-4% at week 7) that continues to deteriorate into week 9(45+/-6%) but recovers by week 10

abnormal response to novel object ( J:126424 )

• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories mice exhibit a decrease in exploration of a novel object

• however, following expression of cre at week 8.5 novel object exploration preference is regained by week 12 and persists through week 30

decreased locomotor activity ( J:126424 )

• at 12 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display decreased activity in an open field compared to Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)37Jcol mice

nervous system

spongiform encephalopathy ( J:126424 )

• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice exhibit evidence of spongiosis in the hippocampus

• however, there is no evidence of neurodegeneration at 6 weeks post-infection and, with the expression of cre beginning at week 8.5, spongiosis is reversed by week 10

abnormal excitatory postsynaptic potential ( J:126424 )

• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display a 50% reduction in excitatory postsynaptic potential (EPSP) compared to uninfected mice or recovered mice

• however, activation of the cre transgene at 8.5 weeks leads to a recovery of EPSP and long term potentiation is normal

immune system

decreased susceptibility to prion infection ( J:126424 )

• mice develop clinical symptoms of prion disease but recover, surviving up to 30 weeks post-infection with scrapie strain Rocky Mountain Laboratories, compared to Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)37Jcol mice that develop clinical symptoms of prion disease at 12 weeks post-infection

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Nefh-cre)22Jcol/Tg(Nefh-cre)22Jcol Tg(Prnp)46Jcol/Tg(Prnp)46Jcol
Genetic Background	involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * FVB

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

nervous system

nervous system phenotype ( J:74652 )

N	• at 18 months of age mice are indistinguishable from wild-type mice with no evidence of neuropathology

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PRNP)45Jcol/Tg(PRNP)45Jcol
Genetic Background	involves: 129 * 129S7/SvEvBrd * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(PRNP)45Jcol mutation (0 available)

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

immune system

increased susceptibility to prion infection ( J:131494 )

• mice are highly susceptible to sporadic CJD, with a 100% attack rate, extremely short and consistent incubation periods

• mice are less susceptible to variant CJD, with only 1/4 developing clinical disease, however all mice show evidence of sub-clinical prion infection

• vCJD-inoculated mice develop abundant PRNP (Prp) plaques, many of the florid type of a central plaque core surrounded by a ring of spongiform vacuoles

• mice are less susceptible to BSE, with no mice developing clinical signs, however 9/12 show evidence of sub-clinical prion infection

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PRNP)35Jcol/Tg(PRNP)35Jcol
Genetic Background	involves: 129 * 129S7/SvEvBrd * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(PRNP)35Jcol mutation (0 available)

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

immune system

increased susceptibility to prion infection ( J:131494 )

• mice are highly susceptible to prions from patients with sporadic Creutzfeldt-Jakob disease (CJD) that contain the codon 129 methionine polymorphism (129MM) but are less susceptible to classical CJD prions from individuals with the codon 129 valine polymorphism (129VV)

• mice are more resistant to variant CJD 129MM prions, with only 1/14 succumbing to clinical disease at a prolonged incubation period, however all mice inoculated with vCJD exhibit pathological and/or biochemical evidence of prion infection and are positive for type 4 PrP^Sc, indicating 100% infection rate

• vCJD-inoculated mice develop abundant PRNP (Prp) plaques, many of the florid type of a central plaque core surrounded by a ring of spongiform vacuoles

• BSE prion inoculated mice develop clinical disease and sub-clinical infection, showing PrP^Sc in the brains of clinically sick and in those not showing clinical signs

• 5/6 of clinically affected and 6/8 of sub-clinically affected BSE-inoculated mice show a distinctive human PrP^Sc type, corresponding to type 2 Prp^Sc seen in sporadic and iatrogenic CJD, and exhibit widespread vacuolation and extensive gliosis consistent with spongiform encephalopathy

nervous system

gliosis ( J:131494 )

spongiform encephalopathy ( J:131494 )

• 4/6 of clinically affected and 6/8 of sub-clinically affected BSE-inoculated mice show a distinctive human PrP^Sc type, corresponding to type 2 Prp^Sc seen in sporadic and iatrogenic CJD, and exhibit widespread vacuolation and extensive gliosis consistent with spongiform encephalopathy

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Sprn^tm1Geno/Sprn^tm1Geno
Genetic Background	involves: 129S2/SvPas * 129S7/SvEvBrd * C57BL/6J * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Sprn^tm1Geno mutation (0 available); any Sprn mutation (12 available)

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

mortality/aging

mortality/aging ( J:184837 )

N	• mice exhibit normal survival unlike Prnp^tm1Cwe homozygotes

behavior/neurological

limb grasping ( J:184837 )

• as in Prnp^tm1Cwe homozygotes

tremors ( J:184837 )

• as in Prnp^tm1Cwe homozygotes

ataxia ( J:184837 )

• as in Prnp^tm1Cwe homozygotes

dysmetria ( J:184837 )

• as in Prnp^tm1Cwe homozygotes

nervous system

nervous system phenotype ( J:184837 )

N	• unlike Prnp^tm1Cwe homozygotes, mice do not exhibit dysmyelination or granulomatous infiltration in the lateral horns of the spinal cord

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PrnpA116VM128V)1309Jama/0
Genetic Background	involves: 129S7/SvEvBrd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp*A116V*M128V)1309Jama mutation (0 available)

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

mortality/aging

premature death ( J:151934 )

• at 176 days

behavior/neurological

impaired righting response ( J:151934 )

ataxia ( J:151934 )

• beginning at 5 months, mice display a widened stance and unsteady gait compared with wild-type mice

• ataxia progresses with time and becomes severe with excessive falling, difficulty righting, and death

abnormal gait ( J:151934 )

• beginning at 5 months, mice display a widened stance and unsteady gait compared with wild-type mice

nervous system

amyloid beta deposits ( J:151934 )

• mice develop amyloid plaques positive for full-length Prnp protein in the neocortex, hippocampus, caudate nucleus, and cerebellar cortex

brain vacuoles ( J:151934 )

• mice exhibit mild scattered vacuolization in the neocortex, hippocampus, thalamus, hypothalamus, caudate nucleus, pons, cerebellum molecular layer, and cerebellum granular layer

homeostasis/metabolism

amyloid beta deposits ( J:151934 )

• mice develop amyloid plaques positive for full-length Prnp protein in the neocortex, hippocampus, caudate nucleus, and cerebellar cortex

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Gerstmann-Straussler-Scheinker syndrome		DOID:4249	OMIM:137440	J:151934

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PrnpS170NN174T)1020Aag/0
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp*S170N*N174T)1020Aag mutation (0 available)

♀	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 )

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Lck-Prnp)33Cwe/0
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Lck-Prnp)33Cwe mutation (1 available)

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

immune system

thymus hypoplasia ( J:129512 )

• at 10.5 weeks

increased CD8-positive, alpha-beta T cell number ( J:129512 )

• the number of immature CD8+ T cells is increased compared to in wild-type mice

increased gamma-delta T cell number ( J:129512 )

• slight

hematopoietic system

thymus hypoplasia ( J:129512 )

• at 10.5 weeks

increased CD8-positive, alpha-beta T cell number ( J:129512 )

• the number of immature CD8+ T cells is increased compared to in wild-type mice

increased gamma-delta T cell number ( J:129512 )

• slight

endocrine/exocrine glands

thymus hypoplasia ( J:129512 )

• at 10.5 weeks

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)C4Cwe/0
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp)C4Cwe mutation (0 available)

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

homeostasis/metabolism

decreased brain copper level ( J:126728 )

• after 12 months, copper levels in the brain fail to increase with age unlike in wild-type mice

• copper levels in the synaptosomes are decreased compared to in wild-type mice

nervous system

decreased brain copper level ( J:126728 )

• after 12 months, copper levels in the brain fail to increase with age unlike in wild-type mice

• copper levels in the synaptosomes are decreased compared to in wild-type mice

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)a20Cwe/0
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp)a20Cwe mutation (2 available)

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

immune system

thymus atrophy ( J:129512 )

decreased thymocyte number ( J:129512 )

• mice exhibit a reduction in thymocyte numbers compared with wild-type mice

• however, supplementation with copper partially reverses lowered thymocyte numbers

decreased DN2 thymocyte number ( J:129512 )

decreased DN4 thymocyte number ( J:129512 )

abnormal T cell differentiation ( J:129512 )

• gammadelta T cell differentiation is promoted while alphabeta T cell differentiation is impaired compared to in wild-type mice

• T cell differentiation is partially arrested at DN3 unlike in wild-type mice

• however, supplementation with copper partially reverses defects in T cell differentiation

increased double-negative T cell number ( J:129512 )

• partially reversed by copper supplementation

increased DN3 thymocyte number ( J:129512 )

decreased double-positive T cell number ( J:129512 )

• partially reversed by copper supplementation

decreased CD4-positive, alpha-beta T cell number ( J:129512 )

decreased CD8-positive, alpha-beta T cell number ( J:129512 )

• slightly

increased gamma-delta T cell number ( J:129512 )

• 9-fold, partially reversed by copper supplementation

abnormal B cell morphology ( J:129512 )

• the proportion of CD19+ and B220+ B cells is increased compared to in wild-type mice

• however, the total number of B cells is normal

homeostasis/metabolism

abnormal copper level ( J:129512 )

• copper levels in the brain and thymus are increased compared to in wild-type mice

increased brain copper level ( J:126728 )

• after 12 months, mice exhibit increased brain copper levels compared with wild-type mice

• copper levels in the synaptosomes are increased compared to in wild-type mice

abnormal magnesium ion homeostasis ( J:126728 )

• mice exhibit an increase in brain magnesium level compared with wild-type mice

hematopoietic system

thymus atrophy ( J:129512 )

decreased thymocyte number ( J:129512 )

• mice exhibit a reduction in thymocyte numbers compared with wild-type mice

• however, supplementation with copper partially reverses lowered thymocyte numbers

decreased DN2 thymocyte number ( J:129512 )

decreased DN4 thymocyte number ( J:129512 )

abnormal T cell differentiation ( J:129512 )

• gammadelta T cell differentiation is promoted while alphabeta T cell differentiation is impaired compared to in wild-type mice

• T cell differentiation is partially arrested at DN3 unlike in wild-type mice

• however, supplementation with copper partially reverses defects in T cell differentiation

increased double-negative T cell number ( J:129512 )

• partially reversed by copper supplementation

increased DN3 thymocyte number ( J:129512 )

decreased double-positive T cell number ( J:129512 )

• partially reversed by copper supplementation

decreased CD4-positive, alpha-beta T cell number ( J:129512 )

decreased CD8-positive, alpha-beta T cell number ( J:129512 )

• slightly

increased gamma-delta T cell number ( J:129512 )

• 9-fold, partially reversed by copper supplementation

abnormal B cell morphology ( J:129512 )

• the proportion of CD19+ and B220+ B cells is increased compared to in wild-type mice

• however, the total number of B cells is normal

nervous system

increased brain copper level ( J:126728 )

• after 12 months, mice exhibit increased brain copper levels compared with wild-type mice

• copper levels in the synaptosomes are increased compared to in wild-type mice

endocrine/exocrine glands

thymus atrophy ( J:129512 )

decreased thymocyte number ( J:129512 )

• mice exhibit a reduction in thymocyte numbers compared with wild-type mice

• however, supplementation with copper partially reverses lowered thymocyte numbers

decreased DN2 thymocyte number ( J:129512 )

decreased DN4 thymocyte number ( J:129512 )

increased DN3 thymocyte number ( J:129512 )

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PrnpD177NM128V)A21Rchi/Tg(PrnpD177NM128V)A21Rchi
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp*D177N*M128V)A21Rchi mutation (1 available)

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

mortality/aging

premature death ( J:142098 )

• mice die from neurological disease with a mean lifespan of around 284 days

• the length of illness (mean day of onset until death) is around 130 days

behavior/neurological

limb grasping ( J:142098 )

• mice suffering form neurological disease tightly clasp hindlimbs upon lifting by tail

ataxia ( J:142098 )

• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset around 144 days

impaired balance ( J:142098 )

• mice develop ataxia with extension of hindlimbs and the unbalanced posture with an age of onset around 144 days

skeleton

kyphosis ( J:142098 )

• kyphosis occurs with the onset of ataxia

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Creutzfeldt-Jakob disease		DOID:11949	OMIM:123400	J:142098

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PrnpD177NM128V)A21Rchi/0
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp*D177N*M128V)A21Rchi mutation (1 available)

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

mortality/aging

premature death ( J:142098 )

• mice die from neurological disease with a mean lifespan of around 704 days

• the length of illness (mean day of onset until death) is around 293 days

behavior/neurological

limb grasping ( J:142098 )

• mice suffering form neurological disease tightly clasp hindlimbs upon lifting by tail

ataxia ( J:142098 )

• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset around 452 days

impaired balance ( J:142098 )

• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset around 452 days

skeleton

kyphosis ( J:142098 )

• kyphosis occurs with the onset of ataxia

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Creutzfeldt-Jakob disease		DOID:11949	OMIM:123400	J:142098

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp-tTA)F959Sbp/0 Tg(tetO-ATXN3)2904Olri/0
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6 * FVB

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp-tTA)F959Sbp mutation (1 available)
Tg(tetO-ATXN3)2904Olri mutation (0 available)

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

nervous system

neuronal intranuclear inclusions ( J:154079 )

• in some neuronal cells of the cerebral cortex by 20 month old mice

behavior/neurological

behavior/neurological phenotype ( J:154079 )

N	• mice exhibit a normal gait and performance on a rotarod

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp-tTA)F959Sbp/Tg(Prnp-tTA)F959Sbp Tg(tetO-ATXN3)2904Olri/0
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6 * FVB

Find Mice

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

behavior/neurological

decreased anxiety-related response ( J:154079 )

• mice spend more time in the inner regions of an open field compared with wild-type mice

abnormal motor capabilities/coordination/movement ( J:154079 )

• some mice exhibit spastic head movements unlike wild-type mice

limb grasping ( J:154079 )

• beginning at 2 to 3 months of age

impaired coordination ( J:154079 )

• when lowered onto a horizontal pen, mice fail to walk along it and fall with severely affected mice unable to sit on the pen unlike wild-type mice

• mice exhibit impaired performance on a rotarod compared with mice containing only one of the transgenes

• however, mice treated with doxycycline exhibit improved rotarod performance

hyperactivity ( J:154079 )

• in an open field test, especially in inner areas

nervous system

Purkinje cell degeneration ( J:154079 )

• at 20 months

neuronal intranuclear inclusions ( J:154079 )

• in some neuronal cells of the cerebral cortex by 1 month old mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Machado-Joseph disease		DOID:1440	OMIM:109150	J:154079

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp-tTA)F959Sbp/0 Tg(tetO-ATXN3)2904Olri/Tg(tetO-ATXN3)2904Olri
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6 * FVB

Find Mice

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

behavior/neurological

decreased anxiety-related response ( J:154079 )

• mice spend more time in the inner regions of an open field than wild-type mice

abnormal motor capabilities/coordination/movement ( J:154079 )

• some mice exhibit spastic head movements unlike wild-type mice

limb grasping ( J:154079 )

• beginning at 2 to 3 months of age

impaired coordination ( J:154079 )

• when lowered onto a horizontal pen, mice fail to walk along it and fall with severely affected mice unable to sit on the pen unlike wild-type mice

• mice exhibit impaired performance on a rotarod compared with mice containing only one of the transgenes

• however, mice treated with doxycycline exhibit improved rotarod performance

abnormal gait ( J:154079 )

• at 20 months, mice exhibit an altered gait with smaller distance between steps compared with wild-type mice

hyperactivity ( J:154079 )

• in an open field test

nervous system

Purkinje cell degeneration ( J:154079 )

• at 20 months

neuronal intranuclear inclusions ( J:154079 )

• in some neuronal cells of the cerebral cortex by 1 month old mice

growth/size/body

decreased body weight ( J:154079 )

• mice exhibit reduced body weight compared with Tg(tetO-ATXN3)2904Olri mice

• however, treatment with doxycycline at 9 weeks of age restores weight

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Machado-Joseph disease		DOID:1440	OMIM:109150	J:154079

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp-tTA)F959Sbp/Tg(Prnp-tTA)F959Sbp Tg(tetO-ATXN3)2904Olri/Tg(tetO-ATXN3)2904Olri
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6 * FVB

Find Mice

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

behavior/neurological

abnormal motor learning ( J:154079 )

• mice exhibit reduced motor learning on a rotarod compared with mice containing only one of the transgenes

decreased anxiety-related response ( J:154079 )

• mice spend more time in the inner regions of an open field compared with wild-type mice

abnormal motor capabilities/coordination/movement ( J:154079 )

• some mice exhibit spastic head movements unlike wild-type mice

limb grasping ( J:154079 )

• beginning at 2 to 3 months of age

impaired coordination ( J:154079 )

• when lowered onto a horizontal pen, mice fail to walk along it and fall with severely affected mice unable to sit on the pen unlike wild-type mice

• as early as 9 weeks of age, mice exhibit impaired performance on a rotarod compared with mice containing only one of the transgenes

• however, mice treated with doxycycline for 5 months exhibit improved rotarod performance

hyperactivity ( J:154079 )

• in an open field test

nervous system

decreased Purkinje cell size ( J:154079 )

• at 21 months, Purkinje cells are smaller than in wild-type mice

• however, mice treated with doxycycline treatment from 2 months of age exhibit normal Purkinje cell size

thin cerebellar molecular layer ( J:154079 )

• at 21 months, the cerebellar molecular layer is thinner than in wild-type mice

• however, mice treated with doxycycline treatment from 2 months of age exhibit normal cerebellar molecular layer thickness

growth/size/body

decreased body weight ( J:154079 )

• mice exhibit reduced body weight compared with Tg(tetO-ATXN3)2904Olri mice

• however, treatment with doxycycline at 9 weeks of age restores weight

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Machado-Joseph disease		DOID:1440	OMIM:109150	J:154079

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe 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:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp*)#Rgab mutation (0 available)

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

mortality/aging

premature death ( J:183170 )

• mice are euthanized when they are too paralyzed to reach food and water

behavior/neurological

limb grasping ( J:183170 )

tremors ( J:183170 )

• in very few mice

hindlimb paralysis ( J:183170 )

• asymmetric hindlimb weakness at 5 to 6 months that progresses to paraplegia

paraparesis ( J:183170 )

• asymmetric hindlimb weakness at 5 to 6 months that progresses to paraplegia

myoclonus ( J:183170 )

• myoclonic jerks in some mice

limbs/digits/tail

abnormal tail morphology ( J:183170 )

• plastic tail in very few mice

muscle

myoclonus ( J:183170 )

• myoclonic jerks in some mice

muscular atrophy ( J:183170 )

• in the lower body

nervous system

myoclonus ( J:183170 )

• myoclonic jerks in some mice

astrocytosis ( J:183170 )

• at 3 months and more prominent at 8 months

neuron degeneration ( J:183170 )

• at 3 months and more prominent at 8 months

skeleton

kyphosis ( J:183170 )

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

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PRNP)23454Sbp/0
Genetic Background	involves: 129S7/SvEvBrd * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(PRNP)23454Sbp mutation (0 available)

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

immune system

increased susceptibility to prion infection ( J:236012 )

• intracerebral inoculation with bovine spongiform encephalopathy (BSE) prions results in prion disease development with a mean incubation period of 210 days

• mice inoculated with brain homogenate from a variant Creutzfeldt-Jakob disease (vCJD) patient results in disease development with a mean incubation period of 199 days

• secondary passage of vCJD prions in mice reduces the mean incubation period to 122 days

• brains of mice inoculated with BSE or vCJD prions exhibit proteinase K-resistant prion protein

• however, none of the mice inoculated with brain homogenate from sporadic CJD patients exhibiting the MM1, MM2, or VV2 disease subtypes develop clinical signs of prion disease or mice inoculated with the RML strain of mouse-passaged scrapie prions

nervous system

nervous system phenotype ( J:236012 )

N	• mice show no signs of spontaneous neurological dysfunction up to 600 days of age

abnormal brain morphology ( J:236012 )

• brains of BSE- and vCJD-inoculated mice exhibit PrP^Sc deposition in the brain

• however, florid PrP^Sc plaques are not seen in the brains of vCJD-inoculated mice

hippocampal neuron degeneration ( J:236012 )

• mice show prominent loss of CA2 and CA3 neurons in the hippocampus upon first passage of BSE prions

• mice show extensive cell loss in all areas of the hippocampus, including the dentate gyrus upon vCJD inoculation

spongiform encephalopathy ( J:236012 )

• brains of BSE- and vCJD-inoculated mice exhibit moderate-to-severe spongiform degeneration and vacuolation upon first passage of the prions

• BSE- and vCJD-inoculated mice show abundant vacuolation in the cerebral cortex

• large clusters of vacuoles and numerous coarse PrP^Sc deposits are seen in the hippocampus upon secondary passage of the vCJD and BSE isolates

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
variant Creutzfeldt-Jakob disease		DOID:5435		J:236012

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)4053Sbp/0
Genetic Background	involves: 129S7/SvEvBrd * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp)4053Sbp mutation (0 available)

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

immune system

increased susceptibility to prion infection ( J:136434 )

• mutants show increased susceptibility to developing scrapie following RML prion inoculation, with an average incubation time of 59 days compared to 138 days in controls and average death of 65 days versus 159 days in controls

• however, scrapie incubation times and time of death in mutants inoculated with RML prions is prolonged by about 10 days compared to single Tg(Prnp)4053Sbp mice

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp*P101L)2866Sbp/0
Genetic Background	involves: 129S7/SvEvBrd * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp*P101L)2866Sbp mutation (0 available)

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

mortality/aging

premature death ( J:136434 )

• mutants die within about 3 days following clinical signs of neurological disease

nervous system

abnormal brain morphology ( J:136434 )

• brains of ill mutants contain abundant prion protein amyloid plaques; prion protein deposits are not resistant to proteinase K digestion

astrocytosis ( J:136434 )

spongiform encephalopathy ( J:136434 )

abnormal nervous system physiology ( J:136434 )

• 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

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(PrnpP101L)2866Sbp/Tg(PrnpP101L)2866Sbp
Genetic Background	involves: 129S7/SvEvBrd * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp*P101L)2866Sbp mutation (0 available)

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

nervous system

spongiform encephalopathy ( J:136434 )

abnormal nervous system physiology ( J:136434 )

• mean age for development of neurologic disease is 85 +/- 2 days

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

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)37Jcol/?
Genetic Background	involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * FVB

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp)37Jcol mutation (0 available)

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

mortality/aging

increased susceptibility to induced morbidity/mortality ( J:74652 )

• mice succumb sooner to scrapies infection at 72+/-5 days compared to 130+/-1 days for wild-type mice

behavior/neurological

abnormal behavior ( J:126424 )

• mice display behavioral defects despite normal grip strength and coordination of movement

• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display reduced burrowing (displacing 66+/-6% of pellets compared to 73+/-4% at week 7) that continues to deteriorate throughout the course of the infection (21%-28% displacement at week 12)

abnormal object recognition memory ( J:126424 )

• while mice behave normally towards a novel object at 7 weeks post-infection with scrapie strain Rocky Mountain Laboratories, at 8 weeks post-infection mice exhibit an inability to recognize a novel object with no preference towards it that lasts through out the course of infection

abnormal response to novel object ( J:126424 )

decreased grooming behavior ( J:126424 )

• by 12 weeks post-infection with scrapie strain Rocky Mountain Laboratories mice exhibit decreased grooming

• however, no other motor signs of prion disease are evident at 12 weeks post-infection

hyperactivity ( J:126424 )

• at 12 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display increased activity in an open field compared to Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Nefh-cre)22Jcol Tg(Prnp)37Jcol mice

nervous system

nervous system phenotype ( J:74652 )

N	• while after hyperpolarization response is absent in Prnp^tm1Cwe homozygotes, mice exhibit a slow after hyperpolarization response similar to that observed in wild-type mice

spongiform encephalopathy ( J:126424 )

• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice exhibit evidence of spongiosis in the hippocampus

• however, at 6 weeks post-infection there is no evidence of neurodegeneration

abnormal excitatory postsynaptic potential ( J:126424 )

• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display a 50% reduction in excitatory postsynaptic potential compared to uninfected mice or recovered mice that deteriorates to 22% of uninfected response by week 9

• however, the amplitude of fiber volleys is linearly related to EPSP as in controls indicating a change in presynaptic action potential and long term potentiation is normal

immune system

increased susceptibility to prion infection ( J:74652 , J:126424 )

• mice succumb sooner to scrapies infection at 72+/-5 days compared to 130+/-1 days for wild-type mice (J:74652)

• mice develop clinical symptoms of prion disease at 12 weeks post-infection with scrapie strain Rocky Mountain Laboratories compared to Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Nefh-cre)22Jcol Tg(Prnp)37Jcol mice that develop clinical symptoms of prion disease but recover, surviving up to 30 weeks post-infection (J:126424)

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)46Jcol/?
Genetic Background	involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * FVB

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp)46Jcol mutation (0 available)

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

nervous system

nervous system phenotype ( J:74652 )

N	• while after hyperpolarization response is absent in Prnp^tm1Cwe homozygotes, mice exhibit a slow after hyperpolarization response similar to that observed in wild-type mice

immune system

immune system phenotype ( J:74652 )

N	• mice succumb to scrapie infection by 122+/-2 days similar to wild-type mice that succumb after 130+/-1 days

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)c35Cwe/0
Genetic Background	Not Specified

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp)c35Cwe mutation (0 available)

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

behavior/neurological

hindlimb paralysis ( J:32214 )

• in some cases, mice not innoculated for scrapie developed paralysis of the hindlimbs as early as 4-6 months after birth

immune system

increased susceptibility to prion infection ( J:32214 )

• scrapie-innoculated transgenic mice displayed typical scrapie symptoms, such as hind limb paresis, foot clasp reflex, kyphosis, ataxia, disorientation and minced gait

• incubation times to occurrence of first symptoms as well as the terminal state were shorter than wild-type control group

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)a19Cwe/0
Genetic Background	Not Specified

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp)a19Cwe mutation (1 available)

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

immune system

increased susceptibility to prion infection ( J:32214 )

• scrapie-innoculated mice displayed typical scrapie symptoms, such as hind limb paresis, foot clasp reflex, kyphosis, ataxia, disorientation and minced gait

• incubation times to occurrence of first symptoms as well as the terminal state were shorter than wild-type control group

Allelic Composition	Prnp^tm1Cwe/Prnp^tm1Cwe Tg(Prnp)a20Cwe/0
Genetic Background	Not Specified

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnp^tm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp)a20Cwe mutation (2 available)

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

immune system

increased susceptibility to prion infection ( J:32214 )

• scrapie-innoculated transgenic mice displayed typical scrapie symptoms, such as hind limb paresis, foot clasp reflex, kyphosis, ataxia, disorientation and minced gait

• incubation times to occurrence of first symptoms as well as the terminal state were shorter than wild-type control group

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