Mouse Genome Informatics
hm
    Dysfprmd/Dysfprmd
A/J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological
• hind-limb clasping develops after 8 months of age
• inability to spread legs when suspended by their tails after 8 months of age

muscle
• perivascular infiltrates and inflammation seen by 8 months of age
• eventually, active myopathy was seen in all skeletal muscles
• endomysial fibrosis at later stages
• abnormalities primarily in proximal muscles at early ages
• hypertrophic fibers, fiber splitting and fat replacement also seen
• fibers with scattered degenerating and regenerating fibers by 4-5 months of age
• increased numbers of necrotic and regenerating fibers with time

homeostasis/metabolism
• endomysial fibrosis at later stages

Mouse Models of Human Disease
OMIM IDRef(s)
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