Mouse Genome Informatics
tg
    Tg(Ckm-GSN*D187N)AJewe/0
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
• by 9 months, mice develop atrophic fibers with basophilic appearance
• at 18 months, mice exhibit atrophic fibers and fibers with internal nuclei, basophilic inclusions, and vacuoles
• at 9 months, mice exhibit scattered muscle fibers with granular blue basophilic appearance indicating degeneration unlike in wild-type mice
• at 7 to 9 months and worse at 12 to 14 months

homeostasis/metabolism
• beginning at 3 months, mice exhibit amyloid depositions around the endomysial capillaries and the surrounding muscle fibers unlike in wild-type mice
• amyloidosis progresses with age
• by 18 months, extensive amyloidosis in the endomysium with occasional punctate deposits within some muscle fibers
• between 9 and 18 months, mice exhibit fibrillar deposits between the sarcolemma and the endomysium unlike in wild-type mice
• at 18 months, intranuclear inclusions are observed in muscle cells

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