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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Ckm-GSN*D187N)AJewe
transgene insertion A, Jeffery W Kelly
MGI:4353799
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(Ckm-GSN*D187N)AJewe/Tg(Ckm-GSN*D187N)AJewe C57BL/6J-Tg(Ckm-GSN*D187N)AJewe MGI:4353803
tg2
Tg(Ckm-GSN*D187N)AJewe/0 C57BL/6J-Tg(Ckm-GSN*D187N)AJewe MGI:4353802


Genotype
MGI:4353803
tg1
Allelic
Composition
Tg(Ckm-GSN*D187N)AJewe/Tg(Ckm-GSN*D187N)AJewe
Genetic
Background
C57BL/6J-Tg(Ckm-GSN*D187N)AJewe
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
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 ID Ref(s)
Amyloidosis, Finnish Type 105120 J:150825




Genotype
MGI:4353802
tg2
Allelic
Composition
Tg(Ckm-GSN*D187N)AJewe/0
Genetic
Background
C57BL/6J-Tg(Ckm-GSN*D187N)AJewe
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
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 ID Ref(s)
Amyloidosis, Finnish Type 105120 J:150825





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last database update
07/12/2016
MGI 6.04
The Jackson Laboratory