Immunological features of fibrodysplasia ossificans progressiva and the dysregulated BMP4 pathway

  • Frederick S. Kaplan
  • Eileen M. Shore
  • Rishi Gupta
  • Paul C. Billings
  • David L. Glaser
  • Robert J. Pignolo
  • Daniel Graf
  • Malek Kamoun
Fibrodysplasia Ossificans Progressiva


Dysregulation of the bone morphogenic protein (BMP)4-signaling pathway can explain many developmental and postnatal features of fibrodysplasia ossificans progressive (FOP). However, many puzzling clinical features of the disease strongly implicate an underlying immunological component to the disorder. In this article, we review features of the BMP4 signaling pathway that regulate the immune system, and suggest an integrated hypothesis for FOP that accounts for both BMP-related abnormalities and immunological features of the condition.

Key Words

Fibrodysplasia ossificans progressiva (FOP) bone morphogenic protein (BMP)4-signaling pathway autoimmunity immune system 


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Copyright information

© Humana Press Inc. 2005

Authors and Affiliations

  • Frederick S. Kaplan
    • 5
    • 1
    • 3
  • Eileen M. Shore
    • 5
    • 2
    • 3
  • Rishi Gupta
    • 5
    • 3
  • Paul C. Billings
    • 5
    • 3
  • David L. Glaser
    • 5
    • 3
  • Robert J. Pignolo
    • 1
    • 3
  • Daniel Graf
    • 4
  • Malek Kamoun
    • 1
    • 3
  1. 1.Department MedicineThe University of Pennsylvania School of MedicinePhiladelphia
  2. 2.Department GeneticsThe University of Pennsylvania School of MedicinePhiladelphia
  3. 3.The Center for Research in FOP and Related DisordersThe University of Pennsylvania School of MedicinePhiladelphia
  4. 4.The Institute of ImmunologyBiomedical Sciences Research Center “Alexander Fleming”VariGreece
  5. 5.Department of Orthopaedic SurgeryThe University of Pennsylvania School of MedicinePhiladelphia

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