Mouse Genome Informatics
involves: 129S4/SvJae * C57BL/6
phenotype observed in females
phenotype observed in males
N normal phenotype
• hind-limb clasping develops after 8 months of age
• inability to spread legs when suspended by their tails after 8 months of age

• perivascular infiltrates and inflammation seen by 8 months of age
• fibers with scattered degenerating and regenerating fibers by 2 months of age
• abnormalities primarily in proximal muscles at early ages
• hypertrophic fibers, fiber splitting and fat replacement are seen at 5 months of age
• nuclei are centrally placed
• by 5 months of age, increased numbers of necrotic and regenerating fibers are present
• endomysial fibrosis at 8 months of age

Mouse Models of Human Disease
Miyoshi Muscular Dystrophy 1; MMD1 254130 J:92838
Muscular Dystrophy, Limb-Girdle, Type 2B; LGMD2B 253601 J:92838
Myopathy, Distal, with Anterior Tibial Onset; DMAT 606768 J:92838