New Publication Characterizes Mouse Model of Calpainopathy

A group of researchers from the Heimer Institute for Muscle Research in Bochum, Germany have characterized a Capn3-deficient mouse strain. This is one of four strains recently generated by Jackson Laboratories as part of a Coalition to Cure Calpain 3 research grant.

This descriptive study analyzed wildtype (WT) and Capn3-deficient (C3) mice of both genders at timepoints between 1.5 months and 15 months. They share the following findings:

  • C3 mice showed similar transcription levels of Capn3 mRNA as WT, but calpain 3 protein expression was almost completely absent.
  • Gastrocnemius, soleus, and psoas muscles from C3 mice showed progressive ultrastructural and histopathological changes. Some differences were found to be gender-specific.
  • C3 mice did not show deficiencies in motor function tests including grip strength and wire hanging tests.

Coalition to Cure Calpain 3 is hopeful that these mice will serve as a tool to help the research community better understand LGMD2A/R1 and how to treat it. We anticipate that this paper will aid in the design of experiments utilizing the C3 strain. The strain has been cryopreserved and is available through the Jackson Labs repository. Additionally, Coalition to Cure Calpain 3 maintains a limited number of live mice. Researchers interested in accessing live mice or the Jackson Labs’ study report should contact C3 Scientific Director Jennifer Levy at jennifer@curecalpain3.org.

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