Anti-Sclerostin抗体[RM0129-4D77] (ab86465)

概述

  • 产品名称
    Anti-Sclerostin抗体[RM0129-4D77]
    参阅全部 Sclerostin 一抗
  • 描述
    大鼠单克隆抗体[RM0129-4D77] to Sclerostin
  • 经测试应用
    适用于: WBmore details
  • 种属反应性
    与反应: Mouse
  • 免疫原

    Recombinant full length protein (Mouse)

性能

  • 形式
    Liquid
  • 存放说明
    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液
    Preservative: None
    Constituents: PBS
  • Concentration information loading...
  • 纯化说明
    IgG fraction of cell culture supernatant purified by Protein A/G affinity chromatography and 0.2 µm filtered.
  • 克隆
    单克隆
  • 克隆编号
    RM0129-4D77
  • 同种型
    IgG2
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab86465 in the following tested applications.

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

应用 Ab评论 说明
WB 1/500 - 1/1000. Predicted molecular weight: 23 kDa.

靶标

  • 功能
    Negative regulator of bone growth.
  • 组织特异性
    Widely expressed at low levels with highest levels in bone, cartilage, kidney, liver, bone marrow and primary osteeoblasts differentiated for 21 days.
  • 疾病相关
    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.
  • 序列相似性
    Belongs to the sclerostin family.
    Contains 1 CTCK (C-terminal cystine knot-like) domain.
  • 细胞定位
    Secreted.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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
    see all

实验方案

文献

ab86465 has not yet been referenced specifically in any publications.

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