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ANKH 抗体 (N-Term)

This anti-ANKH antibody is a 兔 多克隆 antibody detecting ANKH in IHC. Suitable for 人 和 小鼠. This Primary Antibody has been cited in 3+ publications.
产品编号 ABIN965554
发货至: 中国

Quick Overview for ANKH 抗体 (N-Term) (ABIN965554)

抗原

See all ANKH 抗体
ANKH (Ankylosis, Progressive Homolog (Mouse) (ANKH))

适用

  • 19
  • 14
  • 4
  • 2
  • 2
  • 2
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
人, 小鼠

宿主

  • 19

克隆类型

  • 19
多克隆

标记

  • 11
  • 2
  • 2
  • 2
  • 1
  • 1
This ANKH antibody is un-conjugated

应用范围

  • 18
  • 12
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  • 11
  • 3
Immunohistochemistry (IHC)
  • 抗原表位

    • 9
    • 7
    • 4
    • 1
    • 1
    • 1
    N-Term

    纯化方法

    Purified by antigen-specific affinity chromatography.

    免疫原

    Polyclonal antibody produced in rabbits immunizing with a synthetic peptide corresponding to middle residues of human ANKH(Progressive ankylosis protein homolog)
  • 应用备注

    ELISA, Western blotting: 1µg/ml for 2hrs.

    限制

    仅限研究用
  • 状态

    Liquid

    缓冲液

    This antibody is stored in PBS, 50% glycerol

    储存液

    Sodium azide

    注意事项

    This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

    储存条件

    -20 °C
  • Reichenberger, Tiziani, Watanabe, Park, Ueki, Santanna, Baur, Shiang, Grange, Beighton, Gardner, Hamersma, Sellars, Ramesar, Lidral, Sommer, Raposo do Amaral, Gorlin, Mulliken, Olsen: "Autosomal dominant craniometaphyseal dysplasia is caused by mutations in the transmembrane protein ANK." in: American journal of human genetics, Vol. 68, Issue 6, pp. 1321-6, (2001) (PubMed).

    Nürnberg, Thiele, Chandler, Höhne, Cunningham, Ritter, Leschik, Uhlmann, Mischung, Harrop, Goldblatt, Borochowitz, Kotzot, Westermann, Mundlos, Braun, Laing, Tinschert: "Heterozygous mutations in ANKH, the human ortholog of the mouse progressive ankylosis gene, result in craniometaphyseal dysplasia." in: Nature genetics, Vol. 28, Issue 1, pp. 37-41, (2001) (PubMed).

    Ho, Johnson, Kingsley: "Role of the mouse ank gene in control of tissue calcification and arthritis." in: Science (New York, N.Y.), Vol. 289, Issue 5477, pp. 265-70, (2000) (PubMed).

  • 抗原

    ANKH (Ankylosis, Progressive Homolog (Mouse) (ANKH))

    别名

    ANKH

    背景

    The ANKH(Progressive ankylosis protein homolog)regulates intra- and extracellular levels of inorganic pyrophosphate (PPi), probably functioning as PPi transporter. The protein is found in osteoblasts from mandibular bone and from iliac bone, not detected in osteoclastic cells. Defects in ANKH are the cause of craniometaphyseal dysplasia Jackson type (CMDJ). CMDJ is a rare autosomal dominant skeletal disorder characterized by abnormal bone formation and mineralization in membranous as well as endochondral bones. Progressive tickening of the bones can cause narrowing of cranial foramina and can lead to severe visual and neurological impairment, such as facial palsy and deafness.
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