Abstract:
: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.
journal_name
Bonejournal_title
Boneauthors
van Lierop AH,Appelman-Dijkstra NM,Papapoulos SEdoi
10.1016/j.bone.2016.10.010subject
Has Abstractpub_date
2017-03-01 00:00:00pages
51-62eissn
8756-3282issn
1873-2763pii
S8756-3282(16)30300-3journal_volume
96pub_type
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