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Parathyroid hormone-related protein in the aetiology of fibrous dysplasia of bone in the McCune Albright syndrome

Lookup NU author(s): Dr Mark Birch


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OBJECTIVE Fibrous dysplasia, observed in bone lesions in the McCune Albright syndrome (MAS), is thought to result from abnormalities in cells of the osteogenic lineage associated with over-activation of the cAMP signalling pathway in affected cells. The aim of this study was to investigate the role of parathyroid hormone-related protein (PTHrP) in the aetiology of MAS, and to determine a possible therapeutic role for 1,25-dihydroxy vitamin D-3 (1,25(OH)(2)D-3). DESIGN The effects of 1,25(OH)(2)D-3 on PTHrP production and mRNA expression were determined in vitro. 1,25(OH)(2)D-3 therapy was administered to three patients with MAS. PATIENTS Clinical data from four MAS patients (MAS1, 2, 3 and 4), and in vitro studies using bone from three MAS patients (MAS1, 2, and 3), are presented. MEASUREMENTS Immunoradiometric assay and low-cycle number reverse transcriptase-linked PCR were used to determine PTHrP production and mRNA expression in vitro. Standard clinical biochemistry was recorded pre and post commencement of 1,25(OH)(2)D-3 treatment. RESULTS We report the elevated secretion of PTHrP, and a concomitant rise in PTHrP mRNA expression, in cultured osteoblasts from three MAS patients. Treatment with 1,25(OH)(2)D-3 produced a dose-dependent decrease in PTHrP protein secretion and mRNA expression. Marked improvement in bone biochemistry in MAS1, 2 and 3 post treatment with 1,25(OH)(2) D-3 is documented. CONCLUSIONS This study provides the first evidence suggesting that PTHrP may contribute to the aetiology of fibrous dysplasia in MAS. In addition, the therapeutic administration of 1,25(OH)(2)D-3 may provide clinicians with an important new regime for symptomatic relief of bone pain and fracture in some patients with MAS.

Publication metadata

Author(s): Birch MA; Fraser WD; Walsh CA; Durham B; Dillon JP; McCreavy D; Gallagher JA

Publication type: Article

Publication status: Published

Journal: Clinical Endocrinology

Year: 2000

Volume: 53

Issue: 5

Pages: 621-628

ISSN (print): 0300-0664

ISSN (electronic): 1365-2265

Publisher: Wiley-Blackwell Publishing Ltd.


DOI: 10.1046/j.1365-2265.2000.01112.x


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