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Research Article Free access | 10.1172/JCI2361

Reproduction of human fibrous dysplasia of bone in immunocompromised mice by transplanted mosaics of normal and Gsalpha-mutated skeletal progenitor cells.

P Bianco, S A Kuznetsov, M Riminucci, L W Fisher, A M Spiegel, and P G Robey

Department of Experimental Medicine, L'Aquila 67100; University "La Sapienza," Rome 00161, Italy.

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Department of Experimental Medicine, L'Aquila 67100; University "La Sapienza," Rome 00161, Italy.

Find articles by Kuznetsov, S. in: JCI | PubMed | Google Scholar

Department of Experimental Medicine, L'Aquila 67100; University "La Sapienza," Rome 00161, Italy.

Find articles by Riminucci, M. in: JCI | PubMed | Google Scholar

Department of Experimental Medicine, L'Aquila 67100; University "La Sapienza," Rome 00161, Italy.

Find articles by Fisher, L. in: JCI | PubMed | Google Scholar

Department of Experimental Medicine, L'Aquila 67100; University "La Sapienza," Rome 00161, Italy.

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Department of Experimental Medicine, L'Aquila 67100; University "La Sapienza," Rome 00161, Italy.

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Published April 15, 1998 - More info

Published in Volume 101, Issue 8 on April 15, 1998
J Clin Invest. 1998;101(8):1737–1744. https://doi.org/10.1172/JCI2361.
© 1998 The American Society for Clinical Investigation
Published April 15, 1998 - Version history
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Abstract

We have isolated progenitor cells from the stromal system of the fibrous dysplastic marrow of patients with McCune-Albright Syndrome. Analysis of the Gsalpha gene from individual colonies provided direct evidence for the presence of two different genotypes within single fibrous dysplastic lesions: marrow stromal cells containing two normal Gsalpha alleles, and those containing one normal allele and an allele with an activating mutation. Transplantation of clonal populations of normal cells into the subcutis of immunocompromised mice resulted in normal ossicle formation. In contrast, transplantation of clonal populations of mutant cells always led to the loss of transplanted cells from the transplantation site and no ossicle formation. However, transplantation of a mixture of normal and mutant cells reproduced an abnormal ectopic ossicle recapitulating human fibrous dysplasia and providing an in vivo cellular model of this disease. These results provide experimental evidence for the necessity of both normal and mutant cells in the development of McCune-Albright Syndrome fibrous dysplastic lesions in bone.

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