섬유성이형성증 유래세포의 특성연구
CHARACTERISTICS OF FIBROUS DYSPLASIA DERIVED CELLS
1
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Purpose: Fibrous dysplasia (FD) is a fibro-osseous disease associated with activating missense mutations of the gene encoding the α-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
Abstract
Purpose: Fibrous dysplasia (FD) is a fibro-osseous disease associated with activating missense mutations of the gene encoding the α-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
참고문헌 (28)
- Lichtenstein L, "Polyostotic fibrous dysplasia", Arch Surg, 36, pp.874~898 (1938)
- Lichtenstein L, "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, 33, pp.777~816 (1942)
- Chapurlat R, "Current pharmacological treatment for fibrous dysplasia and perspectives for the future", Joint Bone Spine, 72, pp.196~198 (2005)
- Riminucci M, "Fibrous dysplasia as a stem cell disease", J Bone Miner Res, 21(2), pp.125~131 (2006)
- Kos M, "Treatment of monostotic fibrous dysplasia with pamidronate", J Craniomaxillofac Surg, 32, pp.10~15 (2004)
- Lietman SA, "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, 87, pp.2489~2494 (2005)
- Lee JS, "Normal vision despite narrowing of the optic canal in fibrous dysplasia", N Engl J Med, 347, pp.1670~1676 (2002)
- DiCaprio MR, "Fibrous dysplasia. Pathophysiology, evaluation, and treatment", J Bone Joint Surg Am, 87, pp.1848~1864 (2005)
- Weinstein LS, "Activating mutations of the stimulatory G protein in the McCune-Albright syndrome", N Engl J Med, 325, pp.1688~1695 (1991)
- Aldred MA, "Germline mosaicism for a GNAS1 mutation and Albright hereditary osteodystrophy", J Med Genet, 37, pp.35~ (2000)
- Erickson RP, "Somatic gene mutation and human disease other than cancer", Mutat Res, 543, pp.125~136 (2003)
- Karadag A, "A novel technique based on a PNA hybridization probe and FRET principle for quantification of mutant genotype in fibrous dysplasia", McCune-Albright syndrome, 2004, pp.32, 63~
- Idowu BD, "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, 50, pp.691~704 (2007)
- Sakamoto A, "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, 2, pp.67~72 (2000)
- Riminucci M, "A novel GNAS1 mutation, R201G, in McCunealbright syndrome", J Bone Miner Res, 14, pp.1987~1989 (1999)
- Weinstein LS, "G(s)alpha mutations in fibrous dysplasia and McCune-Albright syndrome", J Bone Miner Res, 21(2), pp.120~124 (2006)
- Bianco P, "Diseases of bone and the stromal cell lineage", J Bone Miner Res, 14, pp.336~341 (1999)
- MacDonald-Jankowski DS, "Fibro-osseous lesions of the face and jaws", Clin Radiol, 59, pp.11~25 (2004)
- Corsi A, "Monostotic fibrous dysplasia of the proximal femur and liposclerosing myxofibrous tumor: which one is which?", J Bone Miner Res, 21, pp.1955~1958 (2006)
- Akintoye SO, "Dental characteristics of fibrous dysplasia and McCune- Albright syndrome", Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 96, pp.275~282 (2003)
- Harris WH, "The natural history of fibrous dysplasia. An orthopaedic, pathological, and roentgenographic study", J Bone Joint Surg Am, 44, pp.207~233 (1962)
- Cohen MM, "Etiology of fibrous dysplasia and McCune-Albright syndrome", Int J Oral Maxillofac Surg, 28, pp.366~371 (1999)
- Bianco P, "Reproduction of human fibrous dysplasia of bone in immunocompromised mice by transplanted mosaics of normal and Gsalpha-mutated skeletal progenitor cells", J Clin Invest, 101, pp.1737~1744 (1998)
- Alman BA, "Activating mutations of Gs protein in monostotic fibrous lesions of bone", J Orthop Res, 14, pp.311~315 (1996)
- Riminucci M, "Fibrous dysplasia of bone in the McCune-Albright syndrome: abnormalities in bone formation", Am J Pathol, 151, pp.1587~1600 (1997)
- Collins MT, "Spectrum and natural history of fibrous dysplasia of bone", J Bone Miner Res, 21(2), pp.99~104 (2006)
- Michienzi S, "GNAS transcripts in skeletal progenitors: evidence for random asymmetric allelic expression of Gs alpha", Hum Mol Genet, 16, pp.1921~1930 (2007)
- Bianco P, "Mutations of the GNAS1 gene, stromal cell dysfunction, and osteomalacic changes in non-McCune- Albright fibrous dysplasia of bone", J Bone Miner Res, 15, pp.120~128 (2000)
