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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Prnp-tTA)F959Sbp
transgene insertion F959, Stanley B Prusiner
MGI:2445786
Summary 7 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Prkcetm1Msg/Prkcetm1Msg
Tg(Prnp-tTA)F959Sbp/0
Tg(tetO-Prkce)T9Msg/0
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6J * FVB/N MGI:2651603
cx2
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp-tTA)F959Sbp/0
Tg(tetO-ATXN3)2904Olri/0
involves: 129S7/SvEvBrd * C57BL/6 * FVB MGI:4410602
cx3
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp-tTA)F959Sbp/Tg(Prnp-tTA)F959Sbp
Tg(tetO-ATXN3)2904Olri/0
involves: 129S7/SvEvBrd * C57BL/6 * FVB MGI:4410603
cx4
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp-tTA)F959Sbp/0
Tg(tetO-ATXN3)2904Olri/Tg(tetO-ATXN3)2904Olri
involves: 129S7/SvEvBrd * C57BL/6 * FVB MGI:4410604
cx5
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp-tTA)F959Sbp/Tg(Prnp-tTA)F959Sbp
Tg(tetO-ATXN3)2904Olri/Tg(tetO-ATXN3)2904Olri
involves: 129S7/SvEvBrd * C57BL/6 * FVB MGI:4410605
cx6
Tg(Prnp-tTA)F959Sbp/?
Tg(TetO-Nfkbia*S32A*S36A)1Wcg/?
involves: 129S7/SvEvBrd * DBA * FVB/N MGI:5002518
cx7
Tg(Prnp-tTA)F959Sbp/0
Tg(tetO-Chrnb2*V287L)H3Gica/0
involves: 129S7/SvEvBrd * FVB MGI:3843698


Genotype
MGI:2651603
cx1
Allelic
Composition
Prkcetm1Msg/Prkcetm1Msg
Tg(Prnp-tTA)F959Sbp/0
Tg(tetO-Prkce)T9Msg/0
Genetic
Background
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkcetm1Msg mutation (1 available); any Prkce mutation (39 available)
Tg(Prnp-tTA)F959Sbp mutation (1 available)
Tg(tetO-Prkce)T9Msg mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• in mice administered doxycycline for 2 weeks, restoration of wild-type drinking behavior
• increased duration of regaining ethanol-induced loss or righting response, increase in duration of pentobarbital-induced loss or righting response




Genotype
MGI:4410602
cx2
Allelic
Composition
Prnptm1Cwe/Prnptm1Cwe
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:
Prnptm1Cwe 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
• in some neuronal cells of the cerebral cortex by 20 month old mice

behavior/neurological
N
• mice exhibit a normal gait and performance on a rotarod




Genotype
MGI:4410603
cx3
Allelic
Composition
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp-tTA)F959Sbp/Tg(Prnp-tTA)F959Sbp
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:
Prnptm1Cwe 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
behavior/neurological
• mice spend more time in the inner regions of an open field compared with wild-type mice
• some mice exhibit spastic head movements unlike wild-type mice
• beginning at 2 to 3 months of age
• 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
• in an open field test, especially in inner areas

nervous system
• 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




Genotype
MGI:4410604
cx4
Allelic
Composition
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp-tTA)F959Sbp/0
Tg(tetO-ATXN3)2904Olri/Tg(tetO-ATXN3)2904Olri
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6 * FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnptm1Cwe 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
behavior/neurological
• mice spend more time in the inner regions of an open field than wild-type mice
• some mice exhibit spastic head movements unlike wild-type mice
• beginning at 2 to 3 months of age
• 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
• at 20 months, mice exhibit an altered gait with smaller distance between steps compared with wild-type mice
• in an open field test

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

growth/size/body
• 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




Genotype
MGI:4410605
cx5
Allelic
Composition
Prnptm1Cwe/Prnptm1Cwe
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 Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnptm1Cwe 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
behavior/neurological
• mice exhibit reduced motor learning on a rotarod compared with mice containing only one of the transgenes
• mice spend more time in the inner regions of an open field compared with wild-type mice
• some mice exhibit spastic head movements unlike wild-type mice
• beginning at 2 to 3 months of age
• 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
• in an open field test

nervous system
• 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
• 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
• 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




Genotype
MGI:5002518
cx6
Allelic
Composition
Tg(Prnp-tTA)F959Sbp/?
Tg(TetO-Nfkbia*S32A*S36A)1Wcg/?
Genetic
Background
involves: 129S7/SvEvBrd * DBA * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-tTA)F959Sbp mutation (1 available)
Tg(TetO-Nfkbia*S32A*S36A)1Wcg mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• increased exploratory behavior observed in open and closed arms of plus maze and open field test
• mice exhibit an enhanced spatial learning by locating the visible and hidden platforms of the Morris water maze in less time than controls
• mice exhibit an accelerated retrieval of spatial memory by remaining in target quadrant of probe trial (platform removed from water maze)
• mice are more active than controls
• increased number of rearing events in the open field test
• mice exhibit increased sensitivity to handling-induced seizures

nervous system
• mice exhibit increased sensitivity to handling-induced seizures
• I/O function, a measure of synaptic strength, in untrained mice is significantly impaired relative to controls
• LTP induced in hippocampal slices is higher than controls, however synapse numbers are not increased




Genotype
MGI:3843698
cx7
Allelic
Composition
Tg(Prnp-tTA)F959Sbp/0
Tg(tetO-Chrnb2*V287L)H3Gica/0
Genetic
Background
involves: 129S7/SvEvBrd * FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-tTA)F959Sbp mutation (1 available)
Tg(tetO-Chrnb2*V287L)H3Gica mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• mice exhibit seizures of 3 to 21 episodes during 24 hours each lasting 15 to 40 seconds
• seizures occur mainly during periods of increased delta activity regardless of doxycycline treatment as adults to silence the transgene
• most seizures occur during the light period (resting-sleeping)
• however, doxycycline treatment between E1 and P15 prevents seizures
• mice display abnormal electroencephalogram with frequent spikes of high amplitudes unlike wild-type mice
• delta waves predominant unlike in wild-type mice where theta waves are more representatives
• seizures occur mainly during periods of increased delta activity regardless of doxycycline treatment as adults to silence the transgene
• however, doxycycline treatment between E1 and P15 prevents high amplitude spikes and restores prevalence of theta waves

behavior/neurological
• mice exhibit seizures of 3 to 21 episodes during 24 hours each lasting 15 to 40 seconds
• seizures occur mainly during periods of increased delta activity regardless of doxycycline treatment as adults to silence the transgene
• most seizures occur during the light period (resting-sleeping)
• however, doxycycline treatment between E1 and P15 prevents seizures

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
autosomal dominant nocturnal frontal lobe epilepsy 3 DOID:0060684 OMIM:605375
J:145855





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last database update
04/23/2024
MGI 6.23
The Jackson Laboratory