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- 抗原 See all ANKH 抗体
- ANKH (Ankylosis, Progressive Homolog (Mouse) (ANKH))
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抗原表位
- N-Term
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适用
- 人, 小鼠
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This ANKH antibody is un-conjugated
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应用范围
- 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
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- 应用备注
- ELISA, Western blotting: 1µg/ml for 2hrs.
- 限制
- 仅限研究用
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- 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
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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).
: "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).
: "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).
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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).
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- 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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