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Phenotypes Associated with This Genotype
Genotype
MGI:4440450
Allelic
Composition
Tg(CAG-VCP*A232E)93Jpat/0
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice exhibit decreased survival compared with wild-type mice

skeleton
• mice exhibit increased bone surface to bone volume compared with wild-type mice
• mice exhibit decreased bone volume to total volume compared with wild-type mice
• at 13 months of age, cortical bone density is reduced compared to in wild-type mice
• at 13 months of age, mice exhibit occasional focal areas of thickening cortical bone compared with wild-type mice
• at 13 months of age, trabecular thickness and number are decreased while spacing is increased compared to in wild-type mice
• at 13 months of age

muscle
• disorganized and degenerating
• skeletal muscle fibers exhibit rimmed vacuoles, disorganized sarcomeres, and scattered mitochondria that are enlarged and increased in number unlike in wild-type cells
• at 9 months of age, rimmed vacuoles are present in skeletal muscles unlike in wild-type muscle indicating degeneration
• beginning at 3 to 6 months of age and worsening with age, mice exhibit reduced performance in a hanging wire test compared with wild-type mice
• at 9 months of age, mice exhibit rimmed vacuoles and TDP-43 (Tardbp)+ myopathy unlike wild-type mice

behavior/neurological
• at 16 weeks in an elevated zero maze
• at 14 months, mice exhibit decreased exploration of a novel object after 24 hours of training compared with wild-type mice
• at 16 and 20 weeks, mice exhibit a deficit in hanging wire performance compared with wild-type mice
• beginning at 3 to 6 months of age and worsening with age, mice exhibit reduced performance in a hanging wire test compared with wild-type mice

nervous system
• mild at 14 months
• at 14 months of age, mice exhibit ubiquitin+ and TDP-43 (Tardbp)+ neuropathology unlike wild-type mice

homeostasis/metabolism

growth/size/body
• beginning at 9 months age and continuing as mice age


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
04/19/2016
MGI 6.03
The Jackson Laboratory