Journal of the Korean Association of Oral and Maxillofacial Surgeons

The Korean Association of Oral and Maxillofacial Surgeons (대한구강악안면외과학회)
권호 표지

CHARACTERISTICS OF FIBROUS DYSPLASIA DERIVED CELLS

섬유성이형성증 유래세포의 특성연구

  • Lee, Chan-Hee (School of Dentistry, Seoul National University) ;
  • Han, Ihn (School of Dentistry, Seoul National University) ;
  • Seo, Byoung-Moo (Department of Oral and Maxillofacial Surgery, School of Dentistry, Seoul National University, Dental Research Institute, BK 21)
  • 이찬희 (서울대학교 치의학대학원) ;
  • 한인 (서울대학교 치의학대학원) ;
  • 서병무 (서울대학교 치의학대학원 구강악안면외과, 치학연구소, BK21)
  • Published : 2009.10.30
Download PDF
⟨ Previous Next ⟩

Abstract

Purpose: Fibrous dysplasia (FD) is a fibro-osseous disease associated with activating missense mutations of the gene encoding the $\alpha$-subunit of stimulatory G protein. FD may affect a single bone (called monostotic form) or multiple bones (called polyostotic form). The extent of lesions reflects the onset time of mutation. In this study, cells from monostotic FD in maxilla of a patient were isolated and cultured in vitro for characterization. Materials and Methods: The single cells were released from FD lesion which was surgical specimen from 15 years-old boy. These isolated cells were cultured in vitro and tested their proliferation activity with MTT assay. In osteogenic media, these cells underwent differentiation process comparing with its normal counterpart i.e. bone marrow stromal cells. The proliferated FD cells were detached and transplanted into the dordsal pocket of nude mouse and harvested in 6 weeks and 12 weeks. Results and Summary: FD cells have an increased proliferation rate and poor differentiation. As a result, cells isolated from FD lesion decreased differentiation into osteoblast and increased proliferation capacity. MTT assay presented that proliferation rate of FD cells were higher than control. However, the mineral induction capacity of FD was lesser than that of control. Monostotic FD cells make fewer amounts of bone ossicles and most of them are woven bone rather than lamellar bone in vivo transplantation. In transplanted FD cells, hematopoietic marrow were not seen in the marrow space and filled with the organized fibrous tissue. Therefore, they were recapitulated to the original histological features of FD lesion. Collectively, these results indicated that the FD cells were shown that the increased proliferation and decreased differentiation potential. These in vitro and in vivo system can be useful to test FD cell's fate and possible.

Keywords

References

  1. Lichtenstein L. Polyostotic fibrous dysplasia. Arch Surg 1938;36:874-98 https://doi.org/10.1001/archsurg.1938.01190230153012
  2. Lichtenstein L, Jaffe HL. Fibrous dysplasia of bone: a condition affecting one, several or many bones, the graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism, or still other extraskeletal abnormalities. Arch Pathol 1942;33:777-816
  3. Chapurlat R. Current pharmacological treatment for fibrous dysplasia and perspectives for the future. Joint Bone Spine 2005;72:196-8 https://doi.org/10.1016/j.jbspin.2004.08.001
  4. Riminucci M, Saggio I, Robey PG, Bianco P. Fibrous dysplasia as a stem cell disease. J Bone Miner Res 2006;21 Suppl 2:P125-31 https://doi.org/10.1359/jbmr.06s224
  5. Kos M, Luczak K, Godzinski J, Klempous J. Treatment of monostotic fibrous dysplasia with pamidronate. J Craniomaxillofac Surg 2004;32:10-5 https://doi.org/10.1016/j.jcms.2003.07.009
  6. Lietman SA, Ding C, Levine MA. A highly sensitive polymerase chain reaction method detects activating mutations of the GNAS gene in peripheral blood cells in McCune-Albright syndrome or isolated fibrous dysplasia. J Bone Joint Surg Am 2005;87:2489-94 https://doi.org/10.2106/JBJS.E.00160
  7. Lee JS, FitzGibbon E, Butman JA, Dufresne CR, Kushner H, Wientroub S, et al. Normal vision despite narrowing of the optic canal in fibrous dysplasia. N Engl J Med 2002;347:1670-6 https://doi.org/10.1056/NEJMoa020742
  8. DiCaprio MR, Enneking WF. Fibrous dysplasia. Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 2005;87:1848-64 https://doi.org/10.2106/JBJS.D.02942
  9. Weinstein LS, Shenker A, Gejman PV, Merino MJ, Friedman E, Spiegel AM. Activating mutations of the stimulatory G protein in the McCune-Albright syndrome. N Engl J Med 1991;325:1688-95 https://doi.org/10.1056/NEJM199112123252403
  10. Aldred MA, Bagshaw RJ, Macdermot K, Casson D, Murch SH, Walker-Smith JA, et al. Germline mosaicism for a GNAS1 mutation and Albright hereditary osteodystrophy. J Med Genet 2000;37:E35 https://doi.org/10.1136/jmg.37.11.e35
  11. Erickson RP. Somatic gene mutation and human disease other than cancer. Mutat Res 2003;543:125-36 https://doi.org/10.1016/S1383-5742(03)00010-3
  12. Karadag A, Riminucci M, Bianco P, Cherman N, Kuznetsov SA, Nguyen N, et al. A novel technique based on a PNA hybridization probe and FRET principle for quantification of mutant genotype in fibrous dysplasia/McCune-Albright syndrome. Nucleic Acids Res 2004;32:e63 https://doi.org/10.1093/nar/gnh059
  13. Idowu BD, Al-Adnani M, O'Donnell P, Yu L, Odell E, Diss T, et al. A sensitive mutation-specific screening technique for GNAS1 mutations in cases of fibrous dysplasia: the first report of a codon 227 mutation in bone. Histopathology 2007;50:691-704 https://doi.org/10.1111/j.1365-2559.2007.02676.x
  14. Sakamoto A, Oda Y, Iwamoto Y, Tsuneyoshi M. A comparative study of fibrous dysplasia and osteofibrous dysplasia with regard to Gsalpha mutation at the Arg201 codon: polymerase chain reaction-restriction fragment length polymorphism analysis of paraffin-embedded tissues. J Mol Diagn 2000;2:67-72 https://doi.org/10.1016/S1525-1578(10)60618-6
  15. Riminucci M, Fisher LW, Majolagbe A, Corsi A, Lala R, De Sanctis C, et al. A novel GNAS1 mutation, R201G, in McCunealbright syndrome. J Bone Miner Res 1999;14:1987-9 https://doi.org/10.1359/jbmr.1999.14.11.1987
  16. Weinstein LS. G(s)alpha mutations in fibrous dysplasia and McCune-Albright syndrome. J Bone Miner Res 2006;21 Suppl 2:P120-4 https://doi.org/10.1359/jbmr.06s223
  17. Bianco P, Robey P. Diseases of bone and the stromal cell lineage. J Bone Miner Res 1999;14:336-41 https://doi.org/10.1359/jbmr.1999.14.3.336
  18. MacDonald-Jankowski DS. Fibro-osseous lesions of the face and jaws. Clin Radiol 2004;59:11-25 https://doi.org/10.1016/j.crad.2003.07.003
  19. Corsi A, De Maio F, Ippolito E, Cherman N, Robey GP, Riminucci M, et al. Monostotic fibrous dysplasia of the proximal femur and liposclerosing myxofibrous tumor: which one is which? J Bone Miner Res 2006;21:1955-8 https://doi.org/10.1359/jbmr.060818
  20. Akintoye SO, Lee JS, Feimster T, Booher S, Brahim J, Kingman A, et al. Dental characteristics of fibrous dysplasia and McCune-Albright syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;96:275-82 https://doi.org/10.1016/S1079-2104(03)00225-7
  21. Harris WH, Dudley HR, Jr., Barry RJ. The natural history of fibrous dysplasia. An orthopaedic, pathological, and roentgenographic study. J Bone Joint Surg Am 1962;44-A:207-33
  22. Cohen MM, Jr., Howell RE. Etiology of fibrous dysplasia and McCune-Albright syndrome. Int J Oral Maxillofac Surg 1999;28:366-71 https://doi.org/10.1016/S0901-5027(99)80085-X
  23. Bianco P, Kuznetsov SA, Riminucci M, Fisher LW, Spiegel AM, Robey PG. Reproduction of human fibrous dysplasia of bone in immunocompromised mice by transplanted mosaics of normal and Gsalpha-mutated skeletal progenitor cells. J Clin Invest 1998;101:1737-44 https://doi.org/10.1172/JCI2361
  24. Alman BA, Greel DA, Wolfe HJ. Activating mutations of Gs protein in monostotic fibrous lesions of bone. J Orthop Res 1996;14:311-5 https://doi.org/10.1002/jor.1100140221
  25. Riminucci M, Fisher LW, Shenker A, Spiegel AM, Bianco P, Robey PG. Fibrous dysplasia of bone in the McCune-Albright syndrome: abnormalities in bone formation. Am J Pathol 1997;151:1587-600
  26. Collins MT. Spectrum and natural history of fibrous dysplasia of bone. J Bone Miner Res 2006;21 Suppl 2:P99-P104 https://doi.org/10.1359/jbmr.06s219
  27. Michienzi S, Cherman N, Holmbeck K, Funari A, Collins MT, Bianco P, et al. GNAS transcripts in skeletal progenitors: evidence for random asymmetric allelic expression of Gs alpha. Hum Mol Genet 2007;16:1921-30 https://doi.org/10.1093/hmg/ddm139
  28. Bianco P, Riminucci M, Majolagbe A, Kuzantsov SA, Collins MT, Mankani MH, et al. Mutations of the GNAS1 gene, stromal cell dysfunction, and osteomalacic changes in non-McCune- Albright fibrous dysplasia of bone. J Bone Miner Res 2000;15:120-8 https://doi.org/10.1359/jbmr.2000.15.1.120