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FOP  -  Fibrodysplasia ossificans progressiva

Homo sapiens

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Disease relevance of FOP


Psychiatry related information on FOP


High impact information on FOP


Chemical compound and disease context of FOP


Biological context of FOP


Anatomical context of FOP

  • These findings document mobilization and activation of inflammatory mast cells in the pathology of FOP lesions and provide a novel and previously unrecognized target for pharmacologic intervention in this extremely disabling disease [1].
  • Bone morphogenetic protein-4 (BMP-4) messenger ribonucleic acid (mRNA) and protein are uniquely overexpressed in lymphocytes and lesional cells from patients who have fibrodysplasia ossificans progressiva [2].
  • RESULTS: In the absence of exogenous BMP-4 stimulation (basal state), steady-state levels of all of the BMP antagonists that were investigated were similar in fibrodysplasia ossificans progressiva and control cell lines [2].
  • In the FOP patient group, characteristic anomalies, including large posterior elements, tall narrow vertebral bodies,and fusion of the facet joints between C2 and C7, were observed [4].
  • Given this, it has been suggested that allogenic bone marrow transplantation (BMT) possibly could be a cure for FOP, and drawn attention to a previously unappreciated case of an FOP patient who had successful BMT for aplastic anemia with apparent short- and medium-term arresting of the FOP disease process [13].

Associations of FOP with chemical compounds


Regulatory relationships of FOP


Other interactions of FOP

  • We conclude that mutations in the coding region of noggin are not associated with FOP [11].
  • Bone morphogenetic proteins are potent bone-inducing morphogens that participate in the developmental organization of the skeleton, and increased production of one or more of these proteins has been proposed as the cause of fibrodysplasia ossificans progressiva [7].
  • FOP lymphocytes exhibit defects in the BMP-p38 MAPK pathway, suggesting that altered BMP signaling underlies ectopic bone formation in this disease [20].
  • MSX2, a known Smad pathway target gene, is not upregulated in control or FOP cells in response to BMP, suggesting that lymphocytes do not use this limb of the BMP pathway [20].
  • The FOP phenotype is linked to markers located in the 17q21-22 region (LOD score of 3.41 at the recombination fraction theta = 0) [21].

Analytical, diagnostic and therapeutic context of FOP


  1. Mast cell involvement in fibrodysplasia ossificans progressiva. Gannon, F.H., Glaser, D., Caron, R., Thompson, L.D., Shore, E.M., Kaplan, F.S. Hum. Pathol. (2001) [Pubmed]
  2. Paresis of a bone morphogenetic protein-antagonist response in a genetic disorder of heterotopic skeletogenesis. Ahn, J., Serrano de la Pena, L., Shore, E.M., Kaplan, F.S. The Journal of bone and joint surgery. American volume. (2003) [Pubmed]
  3. A new mutation of the noggin gene in a French Fibrodysplasia ossificans progressiva (FOP) family. Fontaine, K., Sémonin, O., Legarde, J.P., Lenoir, G., Lucotte, G. Genetic counseling (Geneva, Switzerland) (2005) [Pubmed]
  4. Developmental anomalies of the cervical spine in patients with fibrodysplasia ossificans progressiva are distinctly different from those in patients with Klippel-Feil syndrome: clues from the BMP signaling pathway. Schaffer, A.A., Kaplan, F.S., Tracy, M.R., O'Brien, M.L., Dormans, J.P., Shore, E.M., Harland, R.M., Kusumi, K. Spine. (2005) [Pubmed]
  5. The human bone morphogenetic protein 4 (BMP-4) gene: molecular structure and transcriptional regulation. Shore, E.M., Xu, M., Shah, P.B., Janoff, H.B., Hahn, G.V., Deardorff, M.A., Sovinsky, L., Spinner, N.B., Zasloff, M.A., Wozney, J.M., Kaplan, F.S. Calcif. Tissue Int. (1998) [Pubmed]
  6. Corrigendum: A recurrent mutation in the BMP type I receptor ACVR1 causes inherited and sporadic fibrodysplasia ossificans progressiva. Shore, E.M., Xu, M., Feldman, G.J., Fenstermacher, D.A., Brown, M.A., Kaplan, F.S. Nat. Genet. (2007) [Pubmed]
  7. Overexpression of an osteogenic morphogen in fibrodysplasia ossificans progressiva. Shafritz, A.B., Shore, E.M., Gannon, F.H., Zasloff, M.A., Taub, R., Muenke, M., Kaplan, F.S. N. Engl. J. Med. (1996) [Pubmed]
  8. Fibrodysplasia ossificans progressiva (myositis ossificans progressiva) treatment with disodium etidronate. Hall, J.G., Schaller, J.G., Worsham, N.G., Horning, M.R., Staheli, L.T. J. Pediatr. (1979) [Pubmed]
  9. Treatment of patients who have fibrodysplasia ossificans progressiva with isotretinoin. Zasloff, M.A., Rocke, D.M., Crofford, L.J., Hahn, G.V., Kaplan, F.S. Clin. Orthop. Relat. Res. (1998) [Pubmed]
  10. Technetium-99m MDP demonstration of heterotopic ossification in fibrodysplasia ossificans progressiva. Fang, M.A., Reinig, J.W., Hill, S.C., Marini, J., Zasloff, M.A. Clinical nuclear medicine. (1986) [Pubmed]
  11. Linkage exclusion and mutational analysis of the noggin gene in patients with fibrodysplasia ossificans progressiva (FOP). Xu, M.Q., Feldman, G., Le Merrer, M., Shugart, Y.Y., Glaser, D.L., Urtizberea, J.A., Fardeau, M., Connor, J.M., Triffitt, J., Smith, R., Shore, E.M., Kaplan, F.S. Clin. Genet. (2000) [Pubmed]
  12. Fibrodysplasia ossificans progressiva (FOP), a disorder of ectopic osteogenesis, misregulates cell surface expression and trafficking of BMPRIA. de la Peña, L.S., Billings, P.C., Fiori, J.L., Ahn, J., Kaplan, F.S., Shore, E.M. J. Bone Miner. Res. (2005) [Pubmed]
  13. Consideration of Rituximab for fibrodysplasia ossificans progressiva. Altschuler, E.L. Med. Hypotheses (2004) [Pubmed]
  14. Activation of the canonical beta-catenin pathway by histamine. Diks, S.H., Hardwick, J.C., Diab, R.M., van Santen, M.M., Versteeg, H.H., van Deventer, S.J., Richel, D.J., Peppelenbosch, M.P. J. Biol. Chem. (2003) [Pubmed]
  15. Studies on alkaline phosphatase activity cultured cells from a patient with fibrodysplasia ossificans progressiva. Miller, R.L., Maxwell, W.A., Spicer, S.S., Halushka, P.V., Varner, H.H., Westphal, M.C. Lab. Invest. (1977) [Pubmed]
  16. (n-3) fatty acid supplementation in moderately hypertriglyceridemic adults changes postprandial lipid and apolipoprotein B responses to a standardized test meal. Tinker, L.F., Parks, E.J., Behr, S.R., Schneeman, B.O., Davis, P.A. J. Nutr. (1999) [Pubmed]
  17. Fibrodysplasia ossificans progressiva. Biochemical changes in blood serum, urine, skin, bone, and ectopic ossification. Blumenkrantz, N., Asboe-Hansen, G. Scand. J. Rheumatol. (1978) [Pubmed]
  18. Fibrodysplasia ossificans progressiva: current concepts and the role of CT in acute changes. Lindhout, D., Golding, R.P., Taets van Amerongen, A.H. Pediatric radiology. (1985) [Pubmed]
  19. Initiation of fracture repair by bone morphogenetic proteins. Reddi, A.H. Clin. Orthop. Relat. Res. (1998) [Pubmed]
  20. Dysregulation of the BMP-p38 MAPK signaling pathway in cells from patients with fibrodysplasia ossificans progressiva (FOP). Fiori, J.L., Billings, P.C., de la Peña, L.S., Kaplan, F.S., Shore, E.M. J. Bone Miner. Res. (2006) [Pubmed]
  21. Localization of the gene for fibrodysplasia ossificans progressiva (FOP) to chromosome 17q21-22. Lucotte, G., Bathelier, C., Mercier, G., Gérard, N., Lenoir, G., Sémonin, O., Fontaine, K. Genetic counseling (Geneva, Switzerland) (2000) [Pubmed]
  22. Fibrodysplasia (myositis) ossificans progressiva. Bridges, A.J., Hsu, K.C., Singh, A., Churchill, R., Miles, J. Semin. Arthritis Rheum. (1994) [Pubmed]
  23. Anaesthetic management of a child with fibrodysplasia ossificans progressiva. Tumolo, M., Moscatelli, A., Silvestri, G. British journal of anaesthesia. (2006) [Pubmed]
  24. Iatrogenic harm caused by diagnostic errors in fibrodysplasia ossificans progressiva. Kitterman, J.A., Kantanie, S., Rocke, D.M., Kaplan, F.S. Pediatrics (2005) [Pubmed]
  25. Anesthetic management in fibrodysplasia ossificans progressiva (FOP): a case report. Singh, A., Ayyalapu, A., Keochekian, A. Journal of clinical anesthesia. (2003) [Pubmed]
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