– 43% increase in the ratio of glycosylated alpha-dystroglycan (αDG) to total αDG from baseline were measured across all three dosing cohorts, signifying the oral therapy has the potential to address both the root cause of LGMD2i and drive functional improvements for patients
– Average of 70% reduction in creatine kinase (CK), a key marker of muscle breakdown, after 90 days of treatment and average 77% reduction after 180 days
– Increase in velocity in the 10-meter walk test (10MWT) at day 90 and 180, which is an improvement over the decline in velocity seen in the natural history data
– If the development program is successful, BridgeBio believes BBP-418 could be the first approved therapy for the treatment of patients with LGMD2i
– BridgeBio to host investor call on March 14, 2022 at 8:00 AM ET