BPS-804 (setrusumab) is a human monoclonal antibody targeting sclerostin, which is being developed to improve bone strength and thereby reduce fractures in the orphan disease osteogenesis imperfecta (OI).

OI is a rare genetic disorder, commonly known as brittle bone disease, which is characterized by fragile bones that fracture easily. In addition to fractures, individuals with OI often have muscle weakness, hearing loss, fatigue, joint laxity, curved bones, scoliosis, and short stature. The majority of cases of OI (up to 90 %) are caused by a dominant mutation in the genes coding for type I collagen, a key component of healthy bone. Current treatment of OI is based on supportive care, focusing on treating fractures and maximizing mobility. To date, there are no FDA or EMA approved treatments.

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REFERENCES

  1. Based on Osteogenesis Imperfecta Foundation estimates
  2. Based on Orphanet estimates
  3. Shapiro J (2014) Osteogenesis Imperfecta: A Translational Approach to Brittle Bone Disease. Academic Press. Chapter 2: p15-22
  4. Glorieux et al (2017) BPS-804 Anti-Sclerostin Antibody in Adults With Moderate Osteogenesis Imperfecta: Results of a Randomized Phase 2a Trial. JBMR 32: 1496-1504

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