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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(CAG-Ppard*E411P)#Als
transgene insertion, Albert R La Spada
MGI:5897172
Summary 2 genotypes


Genotype
MGI:5897185
cn1
Allelic
Composition
Rgs9tm1.1(cre)Yql/Rgs9+
Tg(CAG-Ppard*E411P)#Als/0
Genetic
Background
B6J.Cg-Rgs9tm1.1(cre)Yql Tg(CAG-Ppard*E411P)#Als
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rgs9tm1.1(cre)Yql mutation (1 available); any Rgs9 mutation (34 available)
Tg(CAG-Ppard*E411P)#Als mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice develop motor abnormalities
• in the cage-ledge test, mice cannot easily dismount from the cage ledge
• mice exhibit a prominent clasping phenotype
• mice exhibit worse latency-to-fall times on the accelerating rotarod
• reduction in combined mesh and grip-strength

nervous system
• reduction in parvalbumin-immunoreactive neurons, indicating a decrease in numbers of striatal neurons
• degeneration of striatal neurons

skeleton




Genotype
MGI:5897176
cn2
Allelic
Composition
Tg(CAG-Ppard*E411P)#Als/0
Tg(Nes-cre)1Kln/0
Genetic
Background
B6J.Cg-Tg(Nes-cre)1Kln Tg(CAG-Ppard*E411P)#Als
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(CAG-Ppard*E411P)#Als mutation (0 available)
Tg(Nes-cre)1Kln mutation (4 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• mice appear smaller by 4-6 months of age and weigh less than controls by 8 months of age

behavior/neurological
• mice exhibit reduced novel-environment exploration
• mice exhibit reduced novel-object recognition
• mice exhibit prominent motor abnormalities by 8 months of age
• in the cage-ledge test, mice cannot easily dismount from the cage ledge
• mice exhibit a prominent clasping phenotype
• mice exhibit worse latency-to-fall times on the accelerating rotarod
• reduction in combined mesh and grip-strength
• decrease in mean stride-length

cellular
• mitochondria in the striatum and cortex are smaller in size
• activities of mitochondrial complex IV and complex V are reduced in the striatum and overall ATP concentration is reduced in the striatum and cortex

nervous system
• brain size of adults is reduced
• reduction in the volume of the basal ganglia
• reduction in the volume of the cortex
• reactive gliosis in the brain
• brain shows reduction in neuron numbers
• reduction in tyrosine hydroxylase-positive dopaminergic neurons in the substantia nigra

skeleton

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Huntington's disease DOID:12858 OMIM:143100
J:233176





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last database update
03/19/2024
MGI 6.23
The Jackson Laboratory