Mouse Genome Informatics
tg1
    Tg(Prnp-TARDBP*A315T)23Jlel/0
involves: C57BL/6 * CD-1 * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• mean survival is 75 days

nervous system
• brainstem neurons exhibit ubiquitin inclusions and astrogliosis unlike in wild-type mice
• in the ventral horn and brainstem neurons
• rare with TARDBP co-localization
• mice exhibit marked astrogliosis compared with wild-type mice
• ventral horn neurons exhibit prominent ubiquitin positive cytoplasmic inclusions unlike in wild-type mice
• however, no pale amorphous cyto-architectural abnormalities observed in early onset severe founders that fail to exhibit germline transmission

behavior/neurological
• mice exhibit late onset, progressive motor defects compared with wild-type mice
• progressive
• progressive

muscle
• quadriceps muscle exhibits atrophy and angular fibers consistent with denervation changes compared with wild-type mice
• in the quadriceps muscle
• in the hindlimb