Sclerostin Recombinant Protein | SOST recombinant protein

Human CellExp Sclerostin; human recombinant

Cat. Num. AAA847073

• Gene Names: SOST; CDD; VBCH; DAND6; SOST1
• Applications: SDS-Page
• Purity: > 95%
• Synonyms: Sclerostin; Human CellExp Sclerostin; human recombinant; SOST; VBCH; SOST recombinant protein

• Host: HEK 293 cells; Source: Human
• Purity/Purification: > 95%
• Form/Format: Lyophilized from 0.22 mum filtered solution in PBS; pH 7.4. Normally Trehalose is added as protectant before lyophilization.
• Sequence Length: 213

• Applicable Applications for SOST recombinant protein: SDS-PAGE
• Endotoxin Level: < 1.0 EU per/mug
• Reconstitution: Reconstitute in sterile deionized water to the desired protein concentration.
• Preparation and Storage: Store at -20 degree C for 12 months.; Centrifuge the vial prior to opening.

Testing Data

Related Product Information for SOST recombinant protein

Sclerostin (SOST) is also known as Sclerosteosis; VBCH; is a secreted glycoprotein with a signal peptide for secretion and a C-terminal cysteine knot-like (CTCK) domain and belongs to the Cerberus/DAN family of bone morphogenetic protein (BMP) antagonists. Sclerostin is produced by the osteocyte and has anti-anabolic effects on bone formation. More recently Sclerostin has been identified as binding to LRP5/6 receptors and inhibiting the Wnt signalling pathway. Wnt pathway inhibition under these circumstances is antagonistic to bone formation (meaning Sclerostin antagonizes bone formation). It has been shown that SOST binds BMP-5; -6; and-7 with high affinity and BMP-2 and-4 with low affinity. Sclerostin production by osteocytes is inhibited by parathyroid hormone; mechanical loading and cytokines including oncostatin M; cardiotrophin-1 and leukemia inhibitory factor. Sclerostin production is increased by calcitonin. Thus; osteoblast activity is self regulated by a negative feedback system. Mutations of Sclerostin is associated with the syndrome Sclerosteosis; and reduced sclerostin expression results in a milder form of the disorder called van Buchem disease.

NCBI and Uniprot Product Information

• NCBI GI #: 13376846
• NCBI GeneID: 50964
• NCBI Accession #: NP_079513.1
• NCBI GenBank Nucleotide #: NM_025237.2
• UniProt Accession #: Q9BQB4; Q495N9
• Molecular Weight: 22.5 kDa
• NCBI Official Full Name: sclerostin
• NCBI Official Synonym Full Names: sclerostin
• NCBI Official Symbol: SOST
• NCBI Official Synonym Symbols: CDD; VBCH; DAND6; SOST1
• NCBI Protein Information: sclerostin
• UniProt Protein Name: Sclerostin
• Protein Family: Sclerostin
• UniProt Gene Name: SOST

NCBI Description

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]

Uniprot Description

SOST: Negative regulator of bone growth that acts through inhibition of Wnt signaling and bone formation. Defects in SOST are the cause of sclerosteosis type 1 (SOST1). 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. Defects in SOST are a cause of van Buchem disease (VBCH). 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. A 52 kb deletion downstream of SOST results in SOST transcription suppression causing van Buchem disease. Defects in SOST are a cause of craniodiaphyseal dysplasia autosomal dominant (CDD). 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. Heterozygous mutations located in the secretion signal of the SOST gene prevent sclerostin secretion and can be responsible for craniodiaphyseal dysplasia. Belongs to the sclerostin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 17q21.31

Cellular Component: extracellular matrix; extracellular region

Molecular Function: protein binding; transcription factor binding

Biological Process: negative regulation of BMP signaling pathway; negative regulation of protein complex assembly; positive regulation of transcription, DNA-dependent; response to mechanical stimulus

Disease: Craniodiaphyseal Dysplasia, Autosomal Dominant; Hyperostosis Corticalis Generalisata; Sclerosteosis 1