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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Prnp-FUS)WT3Cshw
transgene insertion WT3, Christopher Shaw
MGI:5445385
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(Prnp-FUS)WT3Cshw/Tg(Prnp-FUS)WT3Cshw involves: C57BL/6 * Crl:CD1(ICR) MGI:5445435
tg2
Tg(Prnp-FUS)WT3Cshw/0 involves: C57BL/6 * Crl:CD1(ICR) MGI:5445436


Genotype
MGI:5445435
tg1
Allelic
Composition
Tg(Prnp-FUS)WT3Cshw/Tg(Prnp-FUS)WT3Cshw
Genetic
Background
involves: C57BL/6 * Crl:CD1(ICR)
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-FUS)WT3Cshw mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• by 10 to 13 weeks (average survival 82 days)

behavior/neurological
• from 4 weeks, mice exhibit rapid decline in motor function
• by 8 weeks
• at 4 weeks
• from 4 weeks
• by 8 weeks, mice travel less distance in an open field compared with wild-type mice
• slightly stilted at 4 weeks due to hind limb dysfunction
• progressive by 8 weeks

nervous system
• in the anterior horn and white matter tracts of the spinal cord
• in the anterior horn of the spinal cord
• at end-stage, mice exhibit numerous ring-like perinuclear inclusions in layer V neurons of the motor and somatosensory cortices, the insular cortex, the neostriatum and the Purkinje cells of the cerebellum compared with wild-type mice
• however, mice do not exhibit ubiquitinated FTLD-FUS-like inclusions
• mice exhibit fewer motor units in muscles compared with wild-type mice
• ALS-like pathology
• in the anterior horn and white matter tracts of the spinal cord

muscle
• mice exhibit a loss of glycolytic fast-twitch muscle fibers compared with wild-type mice
• at end-stage
• mice exhibit increased fatigue resistance compared with wild-type mice
• mice exhibit weaker twitch and tetanic force compared with wild-type mice

growth/size/body

hematopoietic system
• in the anterior horn and white matter tracts of the spinal cord

immune system
• in the anterior horn and white matter tracts of the spinal cord




Genotype
MGI:5445436
tg2
Allelic
Composition
Tg(Prnp-FUS)WT3Cshw/0
Genetic
Background
involves: C57BL/6 * Crl:CD1(ICR)
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-FUS)WT3Cshw mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• mice exhibit normal survival

immune system
• occasionally in the white matter tracts and anterior horn

nervous system
N
• mice exhibit normal neuron numbers and morphology
• occasionally in the white matter tracts and anterior horn

muscle
N
• mice exhibit normal muscle morphology and physiology

hematopoietic system
• occasionally in the white matter tracts and anterior horn





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last database update
09/13/2016
MGI 6.05
The Jackson Laboratory