Sclerostin (SOST)
Sclerostin
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.
Loss-of-function mutations in
this gene are associated with an autosomal-recessive disorder,
sclerosteosis, which causes progressive bone overgrowth.
A deletion
downstream of this gene, which causes reduced sclerostin expression, is
associated with a milder form of the disorder called van Buchem
disease.
Sclerostin is produced by the osteocyte. These cells originate
from osteoblasts, the cells that form the bone matrix.
Sclerostin has an
inhibitory effect on the life time of the osteoblast. Osteoblasts are
self limiting by a negative feedback system.
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