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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Pcp2-cre)GN135Gsat
transgene insertion GN135, GENSAT Project at Rockefeller University
MGI:3836637
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Psmf1tm1c(EUCOMM)Hmgu/Psmf1tm1c(EUCOMM)Hmgu
Tg(Pcp2-cre)GN135Gsat/0
involves: C57BL/6J * C57BL/6N * FVB/N MGI:6838409


Genotype
MGI:6838409
cn1
Allelic
Composition
Psmf1tm1c(EUCOMM)Hmgu/Psmf1tm1c(EUCOMM)Hmgu
Tg(Pcp2-cre)GN135Gsat/0
Genetic
Background
involves: C57BL/6J * C57BL/6N * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Psmf1tm1c(EUCOMM)Hmgu mutation (0 available); any Psmf1 mutation (13 available)
Tg(Pcp2-cre)GN135Gsat mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• by P60, mice have severely disrupted balance, frequently drag their bodies along the ledge and fall on their heads when lowering themselves into the cage
• older mice exhibit locomotor defects
• mice exhibit an aberrant halting gait and frequently lose their balance by P30 (but not a P21) in the ledge test and gait analysis

hematopoietic system
• reactive microglia become detectable in the deep cerebellar nuclei at P22 and gliosis becomes more severe at P30

immune system
• reactive microglia become detectable in the deep cerebellar nuclei at P22 and gliosis becomes more severe at P30

nervous system
• reactive microglia become detectable in the deep cerebellar nuclei at P22 and gliosis becomes more severe at P30
• a major reduction in Purkinje cell number is seen at 10 months of age
• by P30, Purkinje cell primary and secondary dendrites appear swollen
• by P60, the deep cerebellar nuclei contains large aggregates throughout which are positive for both poly-Ub conjugates and p62
• by P30, a dramatic increase in reactive astrocytes is seen in the DCN, but not the Purkinje or molecular layers
• by P50, some reactive astrocytes are seen in the Purkinje layer
• swellings along the length of the axons in the white matter
• Purkinje cell axon terminals in the deep cerebellar nuclei have swellings
• Purkinje cells lose almost all of their axon terminals in the deep cerebellar nuclei by 10 months of age
• axonal torpedoes, focal swellings on Purkinje cell axons are seen by 10 months of age
• progressive damage to Purkinje cell axon terminals





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last database update
11/12/2024
MGI 6.24
The Jackson Laboratory