Abstract
Animal models of fibrodysplasia ossificans progressiva (FOP) are important for understanding the pathophysiology of FOP and for testing possible therapies. Laboratory-generated genetic animal models, each with features of FOP, provide the opportunity to better understand the biology of FOP, and to study the effectiveness and safety of currently available and emerging therapies.
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Kaplan FS, Shore EM, Connor JM. 2002 Fibrodysplasia ossificans progressiva (FOP). In: Royce PM, Steinmann B, eds. Connective Tissue and Its Heritable Disorders: Molecular, Genetic, and Medical Aspects, 2nd Ed. Wiley-Liss: John Wiley & Sons, New York, pp. 827–840.
Kaplan FS, Glaser DL, Hebela N, Shore EM. 2004 Heterotopic ossification. J Am Acad Orthop Surg 12:116–125.
Valentine BA, Kaplan FS. 1996 Fibrodysplasia ossificans progressiva in cats: a potentially important animal model of the human disease. Feline Practice 24:6.
Kaplan FS, Tabas JA, Zasloff MA. 1990 Fibrodysplasia ossificans progressiva: A clue from the fly?. Calcif Tiss Int 47:117–125.
Kaplan FS, Shore EM. 1996 Bone morphogenetic protein and c-fos: early signals in endochondral bone formation. Bone 19:S513-S521.
Olmsted EA, Gannon FH, Wang Z-Q, et al. 1998 Embryonic overexpression of the c-fos proto-oncogene: a murine stem cell chimera applicable to the study of fibrodysplasia ossificans progressiva in humans. Clin Orthop Rel Res 346:81–94.
Lehmann K, Seemann P, Stricker S, et al. 2003 Mutations in bone morphogenetic protein receptor IB cause brachydactyly type A2. Proc Natl Acad Sci USA 100:12,277–12,282.
Yi SE, Daluiski A, Pederson R, Rosen V, Lyons KM. 2000 The type 1 BMP receptor BMPRIB is required for chondrogenesis in the mouse limb. Development 127:621–630.
Brunet LJ, McMahon JA, McMahon AP, Harland RM. 1998 Noggin, cartilage morphogenesis, and joint formation in the mammalian skeleton. Science 280:1455–1457.
Glaser DL, Economides AN, Wang L, et al. 2003 In vivo somatic cell gene transfer of an engineered noggin mutein prevents BMP4-induced heterotopic ossification. J. Bone Joint Surg 85-A:2332–2342.
Kan L, Hu M, Gomes WA, Kessler JA. 2004 Transgenic mice overexpressing BMP4 develop a fibrody splasia ossificans progressive (FOP)-like phenotype. Am J Pathol 165:1107–1115.
Gannon FH, Valentine BA, Shore EM, Zasloff MA, Kaplan FS. 1998 Acute lymphocytic infiltration in an extremely early lesion of fibrodysplasia ossificans progressiva. Clin Orthop Rel Res 346:19–25.
Urist MR. 1965 Bone formation by autoinduction. Science 150:893–899.
Wozney JM, Rosen V, Celeste AJ, et al. 1988 Novel regulators of bone formation of bone formation: molecular clones and activities. Science 242:1528–1534.
Shafritz AB, Shore EM, Gannon FH, et al. 1996 Overexpression of an osteogenic morphogen in fibrodysplasia ossificans progressiva. N Engl J Med 335:555–561.
Gannon FH, Kaplan FS, Olmsted E, Finkel G, Zasloff MA, Shore EM. 1997 Bone morphogenetic protein 2/4 in early fibromatous lesions of fibrodysplasia ossificans progressiva. Hum Pathol 28:339–343.
Ahn J, Serrano de la Peña L, Shore EM, Kaplan FS. 2003 Paresis of a bone morphogenetic protein-antagonist response in a genetic disorder of heterotopic skeletogenesis. J Bone Joint Surg 85-A:667–674.
Groppe J, Greenwald J, Wiater E, et al. 2202 Structural basis of BMP signaling inhibition by the cystine knot protein Noggin. Nature 420:636–642.
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Kaplan, F.S., Shore, E.M., Pignolo, R.J. et al. Animal models of fibrodysplasia ossificans progressiva. Clinic Rev Bone Miner Metab 3, 229–234 (2005). https://doi.org/10.1385/BMM:3:3-4:229
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DOI: https://doi.org/10.1385/BMM:3:3-4:229