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Article

Fibrodysplasia Ossificans Progressiva (FOP), a Disorder of Ectopic Osteogenesis, Misregulates Cell Surface Expression and Trafficking of BMPRIA

1 Jul 2005

Abstract excerpt

UNLABELLED: FOP is a disorder in which skeletal muscle is progressively replaced with bone. FOP lymphocytes, a model system for exploring the BMP pathway in these patients, exhibit a defect in BMPRIA internalization and increased activation of downstream signaling, suggesting that altered BMP receptor trafficking underlies ectopic bone formation in this disease. INTRODUCTION: Fibrodysplasia ossificans progressiva...

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PMID: 15940369
DOI: 10.1359/jbmr.050305

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