Rabbit SOST Polyclonal Antibody | anti-SOST antibody
SOST, NT (SOST, Sclerostin) (MaxLight 405)
Western Blot (WB)
(Western Blot analysis in mouse liver tissue lysates (35ug/lane) usingMBS643793. SOST (arrow) was detected using the purified Pab.)
Western Blot (WB)
(Western Blot analysis in NCI-H292 cell line lysates (35ug/lane) usingMBS643793. This demonstrates theat MBS643793 detected the SOST protein (arrow).)
Western Blot (WB)
(Western Blot analysis of SOST (arrow) in 293 cell lysates (2 ug/lane) using MBS643793 either nontransfected (Lane 1) or transiently transfected (Lane 2) with the SOST gene.)
Immunohistochemistry (IHC)
(Immunohistochemistry analysis in formalin-fixed and paraffin-embedded human breast carcinoma tissue reacted withMBS643793, which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of MBS643793 for immunohistochemistry.)
Immunohistochemistry (IHC)
(Immunohistochemistry (FFPE) analysis staining of SOST in human liver tissue sections usingMBS643793. Tissue was fixed with formaldehyde and blocked with 3% BSA for 0.5 hour at RT; antigen retrieval was by heat mediation with a citrate buffer (pH 6). Samples were incubated with MBS643793 (1:25) for 1 hours at 37 degree C. A undiluted biotinylated goat polyvalent antibody was used as the secondary antibody.)
NCBI and Uniprot Product Information
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: 17q11.2
Cellular Component: extracellular matrix; Golgi apparatus; proteinaceous extracellular matrix; extracellular space; extracellular region
Molecular Function: heparin binding; protein binding; transcription factor binding
Biological Process: ossification; Wnt receptor signaling pathway; response to mechanical stimulus; positive regulation of transcription, DNA-dependent; negative regulation of ossification; negative regulation of protein complex assembly; negative regulation of BMP signaling pathway
Disease: Sclerosteosis 1; Hyperostosis Corticalis Generalisata; Craniodiaphyseal Dysplasia, Autosomal Dominant
