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Phenotypes Associated with This Genotype
Genotype
MGI:4839655
Allelic
Composition
Tg(Prnp-TARDBP)4Jlel/0
Genetic
Background
involves: C57BL/6 * CD-1 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP)4Jlel mutation (2 available)
phenotype observed in females
phenotype observed in males
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

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 10 DOID:0060201 OMIM:612069
J:165985


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
06/20/2017
MGI 6.10
The Jackson Laboratory