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

Details for Product No. ABIN965554
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Antigen
Synonyms ank, ANK, CCAL2, CMDJ, CPPDD, HANK, MANK, Ank, ankh, wu:fc08d03, Ankh, D15Ertd221e, mKIAA1581
Epitope
N-Term
(12), (8), (2), (2)
Reactivity
Human, Mouse (Murine)
(17), (5), (1), (1), (1), (1), (1), (1)
Host
Rabbit
(18)
Clonality
Polyclonal
Conjugate
Un-conjugated
(1), (1), (1), (1), (1), (1)
Application
Immunohistochemistry (IHC)
(17), (10), (10), (9), (2)
Pubmed 3 references available
Quantity 0.1 mg
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Catalog No. ABIN965554
316.25 $
Plus shipping costs $45.00

Order hotline:

  • +1 404 474 4654
  • +1 888 205 9894 (TF)
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
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: 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).

Nürnberg, Thiele, Chandler et al.: "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).

Reichenberger, Tiziani, Watanabe et al.: "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).

Request Want additional data for this product?

The Independent Validation Initiative strives to provide you with high quality data. Find out more

Catalog No. ABIN965554
316.25 $
Plus shipping costs $45.00

Order hotline:

  • +1 404 474 4654
  • +1 888 205 9894 (TF)
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