Coalition to Cure Calpain 3 (C3) is driving LGMD2A/R1 research by awarding a new grant to Dr. Elisabeth Barton and Dr. Lan Wei-LaPierre of the University of Florida. The project is titled “Strategies to improve calcium handling in LGMD2A/R1.”
LGMD2A/R1 is caused by mutations in the gene for Calpain 3. Researchers do not fully understand the role of Calpain 3 in keeping muscle healthy. Experiments in animal models show one of the consequences of loss of Calpain 3 function is a failure of muscles to adapt to increased exercise. This study aims to clarify how calcium signaling, which is a key regulator of exercise response, is affected when Calpain 3 is absent. Further, Drs. Barton and Wei-LaPierre will use a mouse model of LGMD2A/R1 to test therapeutic strategies that normalize calcium handling in muscle.
“There is intriguing data that demonstrate Calpain 3 is important for muscle to adapt to increased exercise, and that this failure to adapt is central to LGMD2A/R1 pathogenesis,” comments C3 Scientific Director Dr. Jennifer Levy. “This exciting project will help us understand if targeting this pathway through altered calcium handling could be a potential treatment for LGMD2A/R1.”
Drs. Barton and Wei-LaPierre share, “We hope to synergize our collective expertise on calcium handling, muscle function, and signaling pathways to determine if there is a strategy that can enhance adaptation to exercise in LGMD2A/R1 to improve muscle strength without compromising existing functional capacity.”