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Mutations in the DYSF gene, encoding dysferlin, cause muscular dystrophies in man.  We compared 4 dysferlinopathic mouse strains: SJL/J and B10.SJL-Dysfim/AwaJ (B10.SJL), and A/J and B6.A-Dysfprmd/GeneJ (B6.A/J).  The former but not the latter two are overtly myopathic and weaker at 3 months of age.  Following repetitive large-strain injury (LSI) caused by lengthening contractions, all except B6.A/J showed ~40% loss in contractile torque.  Three days later, torque in SJL/J, B10.SJL and controls, but not A/J, recovered nearly completely.  B6.A/J showed ~30% torque loss post-LSI and more variable recovery.  Pre-injury, all dysferlinopathic strains had more centrally nucleated fibers (CNFs) and all but A/J showed more inflammation than controls.  At D3, all dysferlinopathic strains showed increased necrosis and inflammation, but not more CNFs; controls were unchanged.  Dystrophin-null DMDmdx mice showed more necrosis and inflammation than all dysferlin-nulls.  Torque loss and inflammation on D3 across all strains were linearly related to necrosis.  Our results suggest that (1) dysferlin is not required for functional recovery 3 days after LSI; (2) B6.A/J mice recover from LSI erratically; (3) SJL/J and B10.SJL muscles recover rapidly, perhaps due to ongoing myopathy; (4) although they recover function to different levels, all 4 dysferlinopathic strains show increased inflammation and necrosis 3 days after LSI

Distinct Effects of Contraction-Induced InjuryIn Vivoon Four Different Murine Models of Dysferlinopathy