About   Help   FAQ
Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Pcp2-tTA)3Horr
transgene insertion 3, Harry T Orr
MGI:3580157
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Nlgn3tm1Rhn/Y
Tg(Pcp2-tTA)3Horr/0
involves: 129S6/SvEvTac * C57BL/6 * FVB/N MGI:5440732
cx2
Tg(Pcp2-tTA)3Horr/0
Tg(tetO-ATXN1*82Q)#Horr/Tg(tetO-ATXN1*82Q)#Horr
involves: FVB/N MGI:5317102
cx3
Tg(Pcp2-tTA)3Horr/0
Tg(tetO-PRKCG*S361G,-GFP)3Jpka/0
involves: FVB/N MGI:5660030
cx4
Tg(Pcp2-tTA)3Horr/0
Tg(tetO-SPTBN2*)#Lpwr/0
involves: FVB/N MGI:5662096


Genotype
MGI:5440732
cx1
Allelic
Composition
Nlgn3tm1Rhn/Y
Tg(Pcp2-tTA)3Horr/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nlgn3tm1Rhn mutation (1 available); any Nlgn3 mutation (16 available)
Tg(Pcp2-tTA)3Horr mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
N
• mice in the absence of doxycycline (Nlgn3 expression is on) exhibit rescue of the neurological phenotypes seen in Nlgn3 single hemizygotes
• mutants raised in the presence of doxycycline (Nlgn3 expression is off) and then the doxycycline is removed to allow for Nlgn3 reexpression, exhibit removal of ectopic synapses from the distal Purkinje cell dendritic tree




Genotype
MGI:5317102
cx2
Allelic
Composition
Tg(Pcp2-tTA)3Horr/0
Tg(tetO-ATXN1*82Q)#Horr/Tg(tetO-ATXN1*82Q)#Horr
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Pcp2-tTA)3Horr mutation (1 available)
Tg(tetO-ATXN1*82Q)#Horr mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• by 12 weeks of age, but not at 6 weeks of age, mutants start to show signs of ataxia by home cage behavior
• by 32 weeks of age, mutants exhibit overt ataxia
• treatment with doxycycline at 12 or at 32 weeks of age to turn off expression of the gene results in improvement in home cage behavior
• 6 week old mutants exhibit a moderate deficiency on the accelerating rotarod which gets worse at 12 weeks of age and by 32 weeks of age, mutants are unable to stay on the accelerating rotarod
• treatment with doxycycline at 6, 12 or at 32 weeks of age to turn off expression of the gene results in a partial improvement in rotarod performance, but only after an extended period of dox treatment

nervous system
• 6 week old mutants exhibit a mild Purkinje cell atrophy that includes cytoplasmic vacuoles, some dendritic pruning, heterotypic Purkinje and nuclear inclusions
• by 12 and 32 weeks of age, Purkinje cell atrophy is very extensive
• treatment with doxycycline at 6, 12 or at 32 weeks of age to turn off expression of the gene results in improvement of Purkinje cell pathology
• at 12 weeks of age, mutants exhibit a decrease in dendritic arborization and distribution of spines along the remaining dendrites
• treatment with doxycycline at 12 or at 32 weeks of age to turn off expression of the gene results in increased arborization of the Purkinje cell dendritic tree

Mouse Models of Human Disease
OMIM ID Ref(s)
Spinocerebellar Ataxia 1; SCA1 164400 J:95453




Genotype
MGI:5660030
cx3
Allelic
Composition
Tg(Pcp2-tTA)3Horr/0
Tg(tetO-PRKCG*S361G,-GFP)3Jpka/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Pcp2-tTA)3Horr mutation (1 available)
Tg(tetO-PRKCG*S361G,-GFP)3Jpka mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice show a decreased latency to fall off the accelerating rod
• in the walking beam test, mice exhibit an increase in the number of slips of a hind leg per crossing of the beam

nervous system
• at P12, in cerebellum lobule 7, the number of Purkinje cells is reduced and cells are not properly arranged in the Purkinje cell layer but are dispersed in the molecular layer
• at 3 months of age, gaps are seen in the Purkinje cell layer of cerebellum lobule 7, suggesting Purkinje cell loss in this area
• however, mice do not show overt Purkinje cell loss in other areas of the cerebellum
• Purkinje cells exhibit a highly abnormal dendritic tree in organotypic slice cultures; the dendritic tree is reduced in size and branch development and the primary dendritic branches appear thickened and have only short secondary side branches
• juvenile mutants exhibit Purkinje cells with dendritic abnormalities in the area of lobule 7 of the cerebellum, most pronounced at P12 and P18, with mild changes in the area in adults
• at P12, stem dendrites appear thickened and are shortened and the distal dendrites are missing or reduced in size
• at P18, size of dendrites increases, however dendrites are still thickened
• at P28, there is a further increase in dendritic size, but dendrites are still thicker and devoid of small side branches
• at P12, in cerebellum lobule 7, the number of Purkinje cells is reduced

Mouse Models of Human Disease
OMIM ID Ref(s)
Spinocerebellar Ataxia 14; SCA14 605361 J:218354




Genotype
MGI:5662096
cx4
Allelic
Composition
Tg(Pcp2-tTA)3Horr/0
Tg(tetO-SPTBN2*)#Lpwr/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Pcp2-tTA)3Horr mutation (1 available)
Tg(tetO-SPTBN2*)#Lpwr mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice show deficits on the accelerating rotarod at 26 weeks of age but not at earlier time points
• mice exhibit alterations in gait, with increases in both step angle variance and stance width

nervous system
• cerebellar N-acetylaspartate concentrations decline with age
• progressive cerebellar degeneration
• mice exhibit altered metabotropic glutamate receptor 1alpha localization at Purkinje cell dendritic spines
• thinning of the cerebellar molecular layer
• mice show a 35% decrease in the metabotropic glutamate receptor 1alpha-mediated response to parallel fiber stimulation but little change in AMPA responsiveness compared with controls
• the metabotropic glutamate receptor 1alpha-mediated long term potentiation (LTP) is absent
• mice show reduced amplitude and LTP of long-latency patches

Mouse Models of Human Disease
OMIM ID Ref(s)
Spinocerebellar Ataxia 5; SCA5 600224 J:215593





Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
Citing These Resources
Funding Information
Warranty Disclaimer & Copyright Notice
Send questions and comments to User Support.
last database update
07/12/2016
MGI 6.04
The Jackson Laboratory