Anti -Sclerostin, NT (SOST, UNQ2976/PRO7455/PRO7476)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 17; NC_000017.10 (41831099..41836156, complement). Location: 17q11.2

Polyclonal

Rabbit

Recognizes human Sclerostin. Species sequence homology: Mouse.

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, 0.09% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-SOST antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Sclerostin (213aa in rat and human (chr 17q12) and 211aa in mouse) is a novel secreted osteoclast-derived BMP antagonist with unique ligand specificity; it negatively regulates the formation of bone by repressing the differentiation and/or function of osteoblasts induced by BMPs. Since sclerostin expression is confined to the bone resorbing osteoclast, it provides a mechanism whereby bone apposition is inhibited in the vicinity of resorption. Indicating the role of sclerostin in bone remodeling and links bone resorption and bone apposition. Defects in SOST are the cause of sclerosteosis, a progressive sclerosing bone dysplasia.

Antibodies; Abs to Proteins

ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)

Suitable for use in Western Blot, ELISA and Immunohistochemistry.
Dilution: ELISA: 1:1000
Western Blot: 1:50-1:100
Immunohistochemistry (Formalin fixed paraffin embedded): 10ug/ml

24,031 Da[Similar Products]

sclerostin

sclerostin

Sclerostin

SOST_HUMAN

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. [provided by RefSeq, Jul 2008]

Function: Negative regulator of bone growth that acts through inhibition of Wnt signaling and bone formation. Ref.8

Subunit structure: Interacts with LRP4 (via the extracellular domain); the interaction facilitates the inhibition of Wnt signaling. Interacts with LRP5 (via the first two YWTD-EGF repeat domains); the interaction inhibits Wnt-mediated signaling. Interacts with LRP6. Ref.8 Ref.11

Subcellular location: Secreted ? extracellular space ? extracellular matrix Ref.9.

Tissue specificity: Widely expressed at low levels with highest levels in bone, cartilage, kidney, liver, bone marrow and primary osteoblasts differentiated for 21 days. Detected in the subendothelial layer of the aortic intima (at protein level). Ref.9

Involvement in disease: Sclerosteosis 1 (SOST1) [MIM:269500]: An autosomal recessive sclerosing bone dysplasia characterized by a generalized hyperostosis and sclerosis leading to a markedly thickened skull, with mandible, ribs, clavicles and all long bones also being affected. Due to narrowing of the foramina of the cranial nerves, facial nerve palsy, hearing loss and atrophy of the optic nerves can occur. Sclerosteosis is clinically and radiologically very similar to van Buchem disease, mainly differentiated by hand malformations and a large stature in sclerosteosis patients.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.1 Ref.2 Ref.13Van Buchem disease (VBCH) [MIM:239100]: VBCH is an autosomal recessive sclerosing bone dysplasia characterized by endosteal hyperostosis of the mandible, skull, ribs, clavicles, and diaphyses of the long bones. Affected patients present a symmetrically increased thickness of bones, most frequently found as an enlarged jawbone, but also an enlargement of the skull, ribs, diaphysis of long bones, as well as tubular bones of hands and feet. The clinical consequence of increased thickness of the skull include facial nerve palsy causing hearing loss, visual problems, neurological pain, and, very rarely, blindness as a consequence of optic atrophy. Serum alkaline phosphatase levels are elevated.Note: The disease is caused by mutations affecting the gene represented in this entry. A 52 kb deletion downstream of SOST results in SOST transcription suppression causing van Buchem disease. Ref.7Craniodiaphyseal dysplasia autosomal dominant (CDD) [MIM:122860]: A severe bone dysplasia characterized by massive generalized hyperostosis and sclerosis, especially involving the skull and facial bones. The sclerosis is so severe that the resulting facial distortion is referred to as 'leontiasis ossea' (leonine faces) and the bone deposition results in progressive stenosis of craniofacial foramina. Respiratory obstruction due to choanal stenosis compromises the clinical outcomes of affected patients.Note: The disease is caused by mutations affecting the gene represented in this entry. Heterozygous mutations located in the secretion signal of the SOST gene prevent sclerostin secretion and can be responsible for craniodiaphyseal dysplasia. Ref.10

Sequence similarities: Belongs to the sclerostin family.Contains 1 CTCK (C-terminal cystine knot-like) domain.

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