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. Author manuscript; available in PMC: 2014 Aug 1.
Published in final edited form as: Muscle Nerve. 2014 Jul 14;50(2):286–289. doi: 10.1002/mus.24220

Figure 1. ER Stress is Elevated in Dysferlin-deficient A/J Mouse Muscle.

Figure 1

A. Immunoblotting with both Romeo and Hamlet anti-dysferlin antibodies yields discrete bands at ~230 kDa in dysferlin-sufficient A/WySnJ lanes. With longer exposures, faint bands appear in the dysferlin-deficient A/J and B10.SJL lanes with the latter being more prominent. B. Densitometry performed on the ~230 kDa band in the overexposed blots shows a significant difference between A/J and B10.SJL with Hamlet but not Romeo anti-dysferlin (N = 3; Mean ± S.E.M.; *, P < 0.05). C-D. Levels of MG53, annexin-A2, and caveolin-3 are not altered in A/J and B10.SJL muscle (N = 3; Mean ± S.E.M). E-F. The ER stress marker CHOP is elevated several-fold in A/J muscle compared to A/WySnJ and B10.SJL muscle (N = 3; Mean ± S.E.M.; *, P < 0.05). G-H. Representative images of immunogold labeling of dysferlin in ultrathin sections of TA muscle from 2 rats show that dysferlin is enriched away from the triad junctions in membranes close to the Z-disk that are reported to be part of the ER, SR, and Golgi networks. I. Quantitative analyses suggest that dysferlin is enriched in components of the ER, SR, and Golgi networks in mature skeletal muscle (Mean ± S.E.M.; *, P < 0.05).