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Phenotypes Associated with This Genotype
Genotype
MGI:4410605
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


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
04/16/2024
MGI 6.23
The Jackson Laboratory