Mouse Genome Informatics
tg
    Tg(Ckm-GSN*D187N)AJewe/Tg(Ckm-GSN*D187N)AJewe
C57BL/6J-Tg(Ckm-GSN*D187N)AJewe
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
muscle
• between 12 and 18 months, mice exhibit extensive myofiber atrophy with increased numbers of fibers exhibiting vacuoles
• at 12 months, myopathy is severe and includes numerous atrophic fibers, endomysial amylodogenesis, vacuoles, and fatty infiltration
• between 12 and 18 months, mice exhibit extensive myofiber atrophy with increased numbers of fibers exhibiting vacuoles

behavior/neurological
• in a beam traverse challenge, mice exhibit increased time to travel the beam at 7 to 9 and 12 to 14 months, steps taken at 12 to 14 months, and errors per step at 12 to 14 months compared with wild-type mice

homeostasis/metabolism
• muscle amyloidosis is more severe than in mice with only one copy of the transgene and begins as early as 1 month of age
• at 3 months, amyloid deposits are more widespread and extend into the endomysium
• amyloidosis worsens with age
• at 9 to 12 months, intranuclear inclusions are observed in muscle cells
• at 18 months, intranuclear inclusions in muscle cells are more extensive than in mice with only one copy of the transgene

Mouse Models of Human Disease
OMIM IDRef(s)
Amyloidosis, Finnish Type 105120 J:150825