Overview

Description

  • Nature

    Recombinant
  • Source

    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      QGWQAFKNDATEIIPELGEYPEPPPELENNKTMNRAENGGRPPHHPFETK DVSEYSCRELHFTRYVTDGPCRSAKPVTELVCSGQCGPARLLPNAIGRGK WWRPSGPDFRCIPDRYRAQRVQLLCPGGEAPRARKVRLVASCKCKRLTRF HNQSELKDFGTEAARPQKGRKPRPRARSAKANQAELENAY
    • Molecular weight

      23 kDa including tags
    • Amino acids

      24 to 213
    • Tags

      His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab175465 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    Mass Spectrometry

    ELISA

    SDS-PAGE

    Western blot

  • Endotoxin level

    < 1.000 Eu/µg
  • Mass spectrometry

    LC-MS/MS
  • Purity

    > 90 % Densitometry.
    ab175465 is purified using Ni-NTA chromatography.
  • Form

    Lyophilised
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at Room Temperature. Store at +4°C short term (1-2 weeks). Store at -80°C. Avoid freeze / thaw cycle.

    Information available upon request.

  • Reconstitution
    Add 0.1 M acetate buffer pH 4 to prepare a working stock solution of approximately 0.5 mg/mL.

General Info

  • Alternative names

    • BEER
    • CDD
    • Cortical hyperostosis with syndactyly
    • Sclerosteosis
    • Sclerostin
    • Sost
    • SOST_HUMAN
    • SOST1
    • UNQ2976/PRO7455/PRO7476
    • VBCH
    see all
  • Function

    Negative regulator of bone growth.
  • Tissue specificity

    Widely expressed at low levels with highest levels in bone, cartilage, kidney, liver, bone marrow and primary osteeoblasts differentiated for 21 days.
  • Involvement in disease

    Defects in SOST are the cause of sclerosteosis (SOST) [MIM:269500]; also known as cortical hyperostosis with syndactyly. SOST is 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=A 52 kb deletion downstream of SOST results in SOST transcription suppression and is a cause of van Buchem disease (VBCH) [MIM:239100]; also known as hyperostosis corticalis generalisata. 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.
  • Sequence similarities

    Belongs to the sclerostin family.
    Contains 1 CTCK (C-terminal cystine knot-like) domain.
  • Cellular localization

    Secreted.
  • Information by UniProt

Images

  • 12% SDS-PAGE analysis of ab175464

    Lane 1: reduced and boiled sample (2.5 μg)
    Lane 2: non-reduced and non-boiled sample (2.5 μg)

References

ab175465 has not yet been referenced specifically in any publications.

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