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Phenotypes Associated with This Genotype
Genotype
MGI:4839653
Allelic
Composition
Tg(Prnp-TARDBP*A315T)23Jlel/0
Genetic
Background
involves: C57BL/6 * CD-1 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TARDBP*A315T)23Jlel mutation (2 available)
phenotype observed in females
phenotype observed in males
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


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
06/15/2016
MGI 6.04
The Jackson Laboratory