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Phenotypes Associated with This Genotype
Genotype
MGI:5445435
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) (J:189360)
• by 10 to 13 weeks (average survival 82 days) (J:189360)

behavior/neurological
• from 4 weeks, mice exhibit rapid decline in motor function (J:189360)
• from 4 weeks, mice exhibit rapid decline in motor function (J:189360)
• by 8 weeks (J:189360)
• by 8 weeks (J:189360)
• at 4 weeks (J:189360)
• at 4 weeks (J:189360)
• from 4 weeks (J:189360)
• from 4 weeks (J:189360)
• by 8 weeks, mice travel less distance in an open field compared with wild-type mice (J:189360)
• by 8 weeks, mice travel less distance in an open field compared with wild-type mice (J:189360)
• slightly stilted at 4 weeks due to hind limb dysfunction (J:189360)
• slightly stilted at 4 weeks due to hind limb dysfunction (J:189360)
• progressive by 8 weeks (J:189360)
• progressive by 8 weeks (J:189360)

nervous system
• in the anterior horn and white matter tracts of the spinal cord (J:189360)
• in the anterior horn and white matter tracts of the spinal cord (J:189360)
• in the anterior horn of the spinal cord (J:189360)
• in the anterior horn of the spinal cord (J:189360)
• 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 (J:189360)
• however, mice do not exhibit ubiquitinated FTLD-FUS-like inclusions (J:189360)
• 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 (J:189360)
• however, mice do not exhibit ubiquitinated FTLD-FUS-like inclusions (J:189360)
• mice exhibit fewer motor units in muscles compared with wild-type mice (J:189360)
• mice exhibit fewer motor units in muscles compared with wild-type mice (J:189360)
• ALS-like pathology (J:189360)
• ALS-like pathology (J:189360)
• in the anterior horn and white matter tracts of the spinal cord (J:189360)
• in the anterior horn and white matter tracts of the spinal cord (J:189360)

muscle
• mice exhibit a loss of glycolytic fast-twitch muscle fibers compared with wild-type mice (J:189360)
• mice exhibit a loss of glycolytic fast-twitch muscle fibers compared with wild-type mice (J:189360)
• at end-stage (J:189360)
• at end-stage (J:189360)
• mice exhibit increased fatigue resistance compared with wild-type mice (J:189360)
• mice exhibit increased fatigue resistance compared with wild-type mice (J:189360)
• mice exhibit weaker twitch and tetanic force compared with wild-type mice (J:189360)
• mice exhibit weaker twitch and tetanic force compared with wild-type mice (J:189360)

growth/size/body
• from 4 weeks (J:189360)
• from 4 weeks (J:189360)

hematopoietic system
• in the anterior horn and white matter tracts of the spinal cord (J:189360)
• in the anterior horn and white matter tracts of the spinal cord (J:189360)

immune system
• in the anterior horn and white matter tracts of the spinal cord (J:189360)
• in the anterior horn and white matter tracts of the spinal cord (J:189360)


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory