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ANKH 抗体 (N-Term)

ANKH 适用: 人, 小鼠 IHC 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN965554
发货至: 中国
  • 抗原 See all ANKH 抗体
    ANKH (Ankylosis, Progressive Homolog (Mouse) (ANKH))
    抗原表位
    • 9
    • 9
    • 6
    • 1
    • 1
    • 1
    N-Term
    适用
    • 21
    • 13
    • 3
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    人, 小鼠
    宿主
    • 21
    克隆类型
    • 21
    多克隆
    标记
    • 11
    • 2
    • 2
    • 2
    • 2
    • 2
    This ANKH antibody is un-conjugated
    应用范围
    • 20
    • 14
    • 14
    • 14
    • 3
    Immunohistochemistry (IHC)
    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)
    Top Product
    Discover our top product ANKH Primary Antibody
  • 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 (ANKH 产品)
    别名
    ANK antibody, CCAL2 antibody, CMDJ antibody, CPPDD antibody, HANK antibody, MANK antibody, Ank antibody, Ankh antibody, D15Ertd221e antibody, ank antibody, mKIAA1581 antibody, ankh antibody, wu:fc08d03 antibody, wu:fj64g09 antibody, zgc:110290 antibody, ANKH inorganic pyrophosphate transport regulator antibody, progressive ankylosis antibody, ANKH inorganic pyrophosphate transport regulator b antibody, ANKH inorganic pyrophosphate transport regulator S homeolog antibody, ANKH inorganic pyrophosphate transport regulator a antibody, ANKH antibody, Ankh antibody, Ank antibody, ankhb antibody, ankh.S antibody, ankha antibody
    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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