TRPC (transient receptor potential canonical) channels have been implicated as mediators of unregulated calcium influx in dystrophic myofibers or myotubes. 

This transgenic strain overexpresses a dominant-negative form of human TRPC6 (dnTRPC6) cDNA under the control of a modified human ACTA1 promoter. Western blot analysis has demonstrated protein overexpression in all skeletal muscles examined. The development or health of skeletal muscle is not negatively impacted, as assessed by histological methods.

When combined with a mutation that over-expresses TRPC3 in a model of muscular dystrophy (see Stock No. <a href="https://www.jax.org/strain/024052">024052</a>), the dnTRPC6 transgene is found to significantly attenuate all aspects of disease associated with TRPC3 overexpression, including histophathology, the increase in small fiber numbers, and the number of myofibers with central nucleation.

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Mice expressing this dominant negative form of human TRPC6 (dnTRPC6) rescue the muscular dystrophy phenotype of mice overexpressing TRPC3 cDNA under the control of a modified human ACTA1 promoter (Stock No. <a href="https://www.jax.org/strain/024052">024052</a>).

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B6;FVB-Tg(ACTA1-TRPC6*)1Jmol/J Frozen Embryo