ELISA/assay

Human Sclerostin ELISA Kit

MBS760266

48-Strip-Wells

300 USD

ELISA Kit

Human Sclerostin ELISA Kit

[Sclerostin]

[SOST (Sclerostin)/VBCHsclerosteosis]

[sclerostin; Sclerostin; sclerostin; sclerostin]

[SOST]

[SOST; SOST; CDD; VBCH; SOST1]

SOST_HUMAN

Human

213

This assay has high sensitivity and excellent specificity for detection of SOS1. No significant cross-reactivity or interference between SOS1 and analogues was observed.

Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Typical Testing Data/Standard Curve (for reference only)

Samples: Serum, Plasma, Tissue Homogenates And Other Biological Fluids. Assay Type: Sandwich. Detection Range: 0.156-10ng/ml. Sensitivity: <0.094ng/ml

Intra-assay Precision: Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level SOS1 were tested 20 times on one plate, respectively. Intra-Assay: CV<8%. Inter-assay Precision: Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level SOS1 were tested on 3 different plates, 8 replicates in each plate. CV (%) = SD/meanX100. Inter-Assay: CV<10%

Principle of the Assay: This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Anti-SOS1 antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-SOS1 antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and washed with wash buffer. HRP-Streptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the SOS1 amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of SOS1 can be calculated.

13376846

NP_079513.1

NM_025237.2

24,264 Da

Negative Regulation Of TCF-dependent Signaling By WNT Ligand Antagonists Pathway (1269608); Signal Transduction Pathway (1269379); Signaling By Wnt Pathway (1269594); TCF Dependent Signaling In Response To WNT Pathway (1269599); Wnt Signaling Pathway (83061); Wnt Signaling Pathway (471)

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: extracellular matrix; extracellular region; extracellular space; Golgi apparatus; proteinaceous extracellular matrix. Molecular Function: heparin binding; protein binding; transcription factor binding. Biological Process: negative regulation of BMP signaling pathway; negative regulation of ossification; negative regulation of protein complex assembly; ossification; positive regulation of transcription, DNA-dependent; response to mechanical stimulus; Wnt receptor signaling pathway. Disease: Craniodiaphyseal Dysplasia, Autosomal Dominant; Hyperostosis Corticalis Generalisata; Sclerosteosis 1

48-Strip-Wells

300 USD

96-Strip-Wells

415

5x96-Strip-Wells

1825

10x96-Strip-Wells

3425