期刊
BONE
卷 96, 期 -, 页码 51-62出版社
ELSEVIER SCIENCE INC
DOI: 10.1016/j.bone.2016.10.010
关键词
Sclerostin; Sclerosteosis; Van Buchem disease; Bone formation; Bone resorption
资金
- EC [TALOS:Health-F2-2008-201099]
Sclerosteosis and van Buchem disease are two rare bone sclerosing dysplasias caused by genetic defects in the synthesis of sclerostin. In this article we review the demographic, clinical, biochemical, radiological, and histological characteristics of patients with sclerosteosis and van Buchem disease that led to a better understanding of the role of sclerostin in bone metabolism in humans and we discuss the relevance of these findings for the development of new therapeutics for the treatment of patients with osteoporosis. (C) 2016 Elsevier Inc. All rights reserved.
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