Mouse Genome Informatics
tg
    Tg(Prnp-FUS)WT3Cshw/Tg(Prnp-FUS)WT3Cshw
involves: C57BL/6 * Crl:CD1(ICR)
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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

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

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 6, with or without Frontotemporal Dementia; 608030 J:189360