Mouse Genome Informatics
tg1
    Tg(Prnp-TARDBP)4Jlel/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 109 days

muscle
• mice exhibit weakness that manifests as external rotation in one hindlimb followed by development of severe bilateral proximal weakness and hypotonia unlike wild-type mice
• muscles exhibit variation in muscle fiber size, prominent myopathic grouping, and disorganization of the myofiber internal architecture unlike wild-type muscles

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