ANKH antibody (Ankylosis, Progressive Homolog (Mouse)) (N-Term)

Details for Product anti-ANKH Antibody No. ABIN965554, Supplier: Log in to see
Antigen
  • ank
  • ANK
  • CCAL2
  • CMDJ
  • CPPDD
  • HANK
  • MANK
  • Ank
  • ankh
  • wu:fc08d03
  • Ankh
  • D15Ertd221e
  • mKIAA1581
  • ankylosis, progressive homolog (mouse)
  • ANKH inorganic pyrophosphate transport regulator
  • ankylosis, progressive homolog b
  • progressive ankylosis
  • ANKH
  • ankh
  • Ankh
  • ankhb
  • Ank
Epitope
N-Term
16
16
4
2
1
Reactivity
Human, Mouse (Murine)
32
20
3
2
2
2
1
1
1
1
1
1
Host
Rabbit
32
Clonality
Polyclonal
Conjugate
This ANKH antibody is un-conjugated
2
2
2
2
2
2
Application
Immunohistochemistry (IHC)
29
19
16
16
6
1
1
Options
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Immunogen Polyclonal antibody produced in rabbits immunizing with a synthetic peptide corresponding to middle residues of human ANKH(Progressive ankylosis protein homolog)
Purification Purified by antigen-specific affinity chromatography.
Alternative Name ANKH (ANKH Antibody Abstract)
Background 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.
Application Notes ELISA, Western blotting: 1µg/ml for 2hrs.
Restrictions For Research Use only
Format Liquid
Buffer This antibody is stored in PBS, 50% glycerol
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage -20 °C
Product cited in: 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).