Sost/sclerostin potently inhibits bone formation. Studies with genetically modified mice provided insights into the mechanism of action of Sost/sclerostin in bone.

Sclerostin is a protein that in humans is encoded by the SOST gene. [5] It is a secreted glycoprotein with a C-terminal cysteine knot -like (CTCK) domain and sequence similarity to the DAN (differential screening-selected gene aberrative in neuroblastoma) family of bone morphogenetic protein (BMP) antagonists.

Jun 4, 2002 · SOST-related sclerosteosis and SOST-related endosteal hyperostosis, van Buchem type (van Buchem disease) are clinically and radiographically similar disorders of progressive bone overgrowth due to increased bone formation. The disorders differ in severity and in type of molecular genetic variants.

Sclerostin is a small protein expressed by the SOST gene in osteocytes, bone cells that respond to mechanical stress applied to the skeleton and appear to play an important role in the regulation of bone remodeling.

In humans, SOST mutations are associated with inherited high bone mass conditions characterized by excessive bone formation, with a sclerosis phenotype particularly prominent in the skull, jaw and long bones.

In this review, we highlight the current knowledge on the regulation of Sost/sclerotin expression and its mechanism(s) of action, discuss novel observations regarding its role in signaling pathways activated by hormones and mechanical stimuli in bone, and propose future research needed to understand the full potential of therapeutic ...

Jun 1, 2009 · The SOST gene provides instructions for making the protein sclerostin. Sclerostin is produced in osteocytes, which are a type of bone cell. The main function of sclerostin is to stop (inhibit) bone formation.

SOST-related sclerosing bone dysplasia is a disorder of bone development characterized by excessive bone formation (hyperostosis). As a result of hyperostosis, bones throughout the body are denser and wider than normal, particularly the bones of the skull.

Mar 5, 2016 · The SOST gene is located at 17q12-21, and codes for the protein sclerostin. Sclerostin is made primarily by osteocytes, and it inhibits bone formation and enhances apoptosis of osteoblasts. Patients with mutations in the SOST gene have very high bone density. SOST binds to LRP5 and inhibits the Wnt-signalling pathway. It is also a mild BMP ...

Mar 1, 2017 · Genetic and pharmacologic inhibition of Sost/sclerostin protects the skeleton by stimulating bone formation and inhibiting bone resorption.