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

The Journal of Clinical Investigation
P BiancoP G Robey

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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Citations

Oct 6, 1999·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·P G Robey, P Bianco
Dec 18, 2007·Der Orthopäde·R Rödl, C Götze
Jun 13, 2008·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·A DumasD Chappard
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Jul 15, 1999·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·R P StantonV L Funanage
Jan 26, 2000·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·P BiancoP G Robey
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Sep 16, 2010·Journal of Molecular Endocrinology·Mara RiminucciPaolo Bianco
Nov 20, 2013·Clinical Orthopaedics and Related Research·Ernesto IppolitoMichael T Collins
Aug 26, 2014·Annual Review of Cell and Developmental Biology·Paolo Bianco

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