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Phenotypes Associated with This Genotype
Genotype
MGI:5556065
Allelic
Composition
Fkrptm1Scbr/Fkrptm1Scbr
Sox1tm1(cre)Take/Sox1+
Tg(CAG-LARGE)126Fmu/0
Genetic
Background
involves: C57BL/6NCrlj * C57BL/10 * CBA/Ca * CBA/JNCrlj
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fkrptm1Scbr mutation (1 available); any Fkrp mutation (13 available)
Sox1tm1(cre)Take mutation (1 available); any Sox1 mutation (1 available)
Tg(CAG-LARGE)126Fmu mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• poor health results in sacrifice at around 27 weeks of age

muscle
• individual muscle fibers are occasionally surrounded by moderate to large amounts of compact, fibrous connective tissue and infiltrates of fat with some muscle fibers mineralized
• mice surviving to 30 weeks exhibit hypertrophy of muscle fibers
• presence of large calcium deposits in muscle
• mice display more severe muscle phenotype than Fkrptm1Scbr Sox1tm1(cre)Take mice
• variation in fiber size, centrally nucleated muscle fibers and increases in the number of split fibers at 12 weeks of age
• pronounced expansion of the interstitium with small to moderate amounts of variably mature fibroadipose tissue and substantial inflammation in the interstitium and necrotic muscle fibers comprised of neutrophils, macrophages, and lesser numbers of lymphocytes and plasma cells
• muscle shows a reduction in resistance to eccentric contraction-induced injury (isometric force in the last contraction) relative to controls
• substantial inflammation in the interstitium and necrotic muscle fibers comprised of neutrophils, macrophages, and lesser numbers of lymphocytes and plasma cells

behavior/neurological
• partial collapse of the leg extensor reflex

immune system
• substantial inflammation in the interstitium and necrotic muscle fibers comprised of neutrophils, macrophages, and lesser numbers of lymphocytes and plasma cells


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
07/16/2019
MGI 6.14
The Jackson Laboratory