ELISA/assay

Rat SOST (Sclerostin) ELISA Kit

MBS2516209

24-Strip-Wells

215 USD

ELISA Kit

Rat SOST (Sclerostin) ELISA Kit

[SOST]

[sclerostin; Sclerostin; sclerostin; sclerostin]

[SOST]

[SOST; SOST; CDD; VBCH; SOST1]

SOST_HUMAN

Rat

213

This kit recognizes natural and some recombinant Rat SOST. No significant crossreactivity or interference between Rat SOST and analogues was observed.

Store at 4 degree C.

Typical Testing Data/Standard Curve (for reference only)

Samples: Serum, Plasma, Biological Fluids. Assay Type: Sandwich. Detection Range: 15.625-1000pg/mL. Sensitivity: Min: 9.375pg/mL; Max: 1000pg/mL

Intended Uses: This ELISA kit can be applied to the in vitro quantitative determination of Rat SOST concentrations in serum, plasma and other biological fluids. Principle of the Assay: This ELISA kit uses Sandwich-ELISA as the method. The micro ELISA plate provided in this kit has been precoated with an antibody specific to Rat SOST. Standards or samples are added to appropriate micro ELISA plate wells and combined with the specific antibody. Then a biotinylated detection antibodies specific for Rat SOST and Avidin-Horseradish Peroxidase (HRP) conjugate are added to each micro plate well successively and incubated. After incubation, free components are washed away. Then the Substrate Reagent is added to each well, only those wells that contain Rat SOST, biotinylated detection antibody and Avidin-HRP conjugate will appear blue in color. The enzyme-substrate reaction will be terminated by adding Stop Solution and appears yellow in color. The optical density (OD) can be measured with spectrophotometry at a wavelength of 450 nm ± 2 nm. The OD value is proportional to the concentration of Rat SOST. The concentration of Rat SOST in samples can be calculated by comparing the OD of the samples with the standard curve.

13376846

NP_079513.1

NM_025237.2

24,264 Da

Disease Pathway (530764); RNF Mutants Show Enhanced WNT Signaling And Proliferation Pathway (1127632); Signal Transduction Pathway (477114); Signaling By WNT In Cancer Pathway (1127614); Signaling By Wnt Pathway (106510); TCF Dependent Signaling In Response To WNT Pathway (1127516); Wnt Signaling Pathway (83061); Wnt Signaling Pathway (471); XAV939 Inhibits Tankyrase, Stabilizing AXIN Pathway (1127633); Misspliced LRP5 Mutants Have Enhanced Beta-catenin-dependent Signaling Pathway (1127631)

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]

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, signal peptide; Secreted. Chromosomal Location of Human Ortholog: 17q11.2. Cellular Component: Golgi apparatus; extracellular matrix; extracellular space; proteinaceous extracellular matrix; 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

24-Strip-Wells

215 USD

48-Strip-Wells

410

96-Strip-Wells

490

5x96-Strip-Wells

2040

10x96-Strip-Wells

3590