The distribution and characterization of skeletal muscle lesions in dysferlin-deficient SJL and A/J mice

K Kobayashi, T Izawa, M Kuwamura… - … and Toxicologic Pathology, 2010 - Elsevier
The pathogenesis of limb-girdle muscular dystrophy type 2B (LGMD2B) dysferlinopathy
remains to be investigated. The distribution and characterization of skeletal muscle lesions
were examined in two different LGMD2B mouse models, SJL and A/J mice (at 10 and 35
weeks old), in association with the endoplasmic reticulum (ER) stress. SJL mice showed an
earlier age of onset and a faster progression of skeletal muscle lesions as compared with
those of A/J mice; the sensitivity difference to muscular dystrophic lesions between SJL and …