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Immunological features of fibrodysplasia ossificans progressiva and the dysregulated BMP4 pathway |
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| Authors: | Frederick S. Kaplan MD Eileen M. Shore PhD Rishi Gupta MD Paul C. Billings PhD David L. Glaser Robert J. Pignolo MD PhD Daniel Graf PhD Malek Kamoun MD |
| Affiliation: | (1) Department Medicine, The University of Pennsylvania School of Medicine, Philadelphia, PA;(2) Department Genetics, The University of Pennsylvania School of Medicine, Philadelphia;(3) The Center for Research in FOP and Related Disorders, The University of Pennsylvania School of Medicine, Philadelphia, PA;(4) The Institute of Immunology, Biomedical Sciences Research Center “Alexander Fleming”, Vari, Greece;(5) Department of Orthopaedic Surgery, The University of Pennsylvania School of Medicine, Silverstein Two, 3400 Spruce St., 19104 Philadelphia, PA |
| Abstract: | 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. |
| Keywords: | Fibrodysplasia ossificans progressiva (FOP) bone morphogenic protein (BMP)4-signaling pathway autoimmunity immune system |
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