Anti-SOST Antibody

$99.00$319.00

Reactivity: Human
Applications: ELISA,IHC
Conjugation: Various
Supplier: CUSABIO BIOTECH CO.

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Rabbit Anti-SOST Antibody (CSB-PA858415ESR1HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

SOST Antibody

More alternative names for the antibody
BEER antibody|CDD antibody|Cortical hyperostosis with syndactyly antibody|Sclerosteosis antibody|Sclerostin antibody|Sost antibody|SOST_HUMAN antibody|SOST1 antibody|UNQ2976/PRO7455/PRO7476 antibody|VBCH antibody
Anti-Sclerostin antibody (ab63097)
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Recommended applications: ELISA, IHC

Recommended dilution: Recommended dilution:IHC:1:20-1:200

Recommended protocols: check protocols

Name

Anti-SOST Antibody

Catalogue No.

CSB-PA858415ESR1HU

Reactivity

Human

Immunogen

Recombinant human Sclerostin protein (24-213AA)

Host

Rabbit

Applications

ELISA, IHC

Recommended dilution

Recommended dilution:IHC:1:20-1:200

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

24 kDa

Purification

Antigen Affinity Purified

Storage

Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.

Database links

Entrez Gene:50964( Human), Entrez Gene:74499( Mouse), Entrez Gene:80722( Rat), Omim:605740( Human), SwissProt:Q9BQB4( Human), SwissProt:Q99P68( Mouse), SwissProt:Q99P67( Rat), Unigene:349204( Human), Unigene:265602( Mouse), Unigene:95369( Rat)

Protein function

Negative regulator of bone growth that acts through inhibition of Wnt signaling and bone formation. .

Protein 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). .

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.; Van 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.; Craniodiaphyseal 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.

Protein sequence and domain

Belongs to the sclerostin family.

Protein cellular localization

Secreted, extracellular space, extracellular matrix .

Research area

All research areas>Signaling Intermediates>Sclerostin
(View all antibody categories related to Signaling Intermediates)

Note

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Supplier

CUSABIO BIOTECH CO.

Product type

Primary antibody

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