For Research Use Only. Not for use in diagnostic procedures.
Sclerostin is the protein product of the SOST gene, which is located at 17q12-21 and highly conserved across vertebrate species. The highest expression of sclerostin throughout the ***** skeleton has been observed in hypertrophic chondrocytes and osteocytes. Sclerostin blocks canonical Wnt signaling by binding to the Wnt coreceptors LRP5/6, inhibiting bone formation by regulating osteoblast function and promoting osteoblast apoptosis. Sclerostin also antagonizes bone morphogenetic protein (BMP) action (e.g. osteoblast differentiation), but does not inhibit direct BMP-induced responses. Sclerostin expression is down-regulated by Parathyroid hormone (PTH), as well as, by the mechanical stimulation of bone. Reduced expression of sclerostin can result in van Buchem disease, while a complete absence results in Sclerosteosis. Patients affected by Sclerosteosis show progressive hyperostosis and sclerosis of the skull, mandible and all long bones. Bone mineral density (BMD), bone volume, bone formation rate, and bone strength are significantly increased, while overall skeletal morphology appears to be normal. A predominance of sclerostin causes reduced bone quality (Osteoporosis pseudoglioma (OPPG) syndrome). Down-regulation of sclerostin might be used as a treatment for diseases such as osteoporosis, promote osseointegration of implants, prevent periprosthetic bone loss, or treat non-union in fractures. Local enhancement of sclerostin expression might be used to prevent cancer metastasis and minimize further expansion of ectopic bone formation.
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Sclerosteosis
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Van Buchem disease
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Osteoporosis pseudoglioma (OPPG) syndrome
An enzyme immunoassay for the quantitation of sclerostin in human serum.