Mouse Genome Informatics
tg
    Tg(Camk2a-Tardbp)#Ckjs/Tg(Camk2a-Tardbp)#Ckjs
FVB/N-Tg(Camk2a-Tardbp)#Ckjs
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• mean survival is 495 days compared to 632 days for wild-type mice

nervous system
• at 6 months, hippocampus volume is decreased compared to in wild-type mice
• at 6 months
• at 6 months, ubiquitin+ inclusions are detected in the neurons unlike in wild-type cells
• however, no inclusions are observed at 2 months
• cortical neurons are reduced in number compared to in wild-type mice
• at 2 months, mice exhibit decreased miniature excitatory postsynaptic current (mEPSC) amplitude and altered decay kinetics compared to in wild-type mice
• at 6 months, mice exhibit neuronal loss that resembles frontotemporal lobar degeneration with ubiquitin+ inclusions unlike wild-type mice

behavior/neurological
• at 2 and 6 months, mice exhibit impaired 1-hour novel object recognition compared with wild-type mice
• at 2 months, mice exhibit impaired performance in a Morris water maze compared to wild-type mice
• at 6 months, mice develop progressive motor behavioral deficits unlike wild-type mice
• at 6 months
• at 6 months on a rotarod

cellular

Mouse Models of Human Disease
OMIM IDRef(s)
Frontotemporal Lobar Degeneration with Tdp43 Inclusions, Grn-Related 607485 J:163612