Sclerostin deficiency in humans.

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

Bone

journal_title

Bone

authors

van Lierop AH,Appelman-Dijkstra NM,Papapoulos SE

doi

10.1016/j.bone.2016.10.010

subject

Has Abstract

pub_date

2017-03-01 00:00:00

pages

51-62

eissn

8756-3282

issn

1873-2763

pii

S8756-3282(16)30300-3

journal_volume

96

pub_type

杂志文章,评审

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