ANKH antibody (N-Term)

Catalog No. ABIN965554

Product Details anti-ANKH Antibody

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

Binding Specificity: N-Term

Reactivity: Human, Mouse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This ANKH antibody is un-conjugated

Application: Immunohistochemistry (IHC)

Purification: Purified by antigen-specific affinity chromatography.

Immunogen: Polyclonal antibody produced in rabbits immunizing with a synthetic peptide corresponding to middle residues of human ANKH(Progressive ankylosis protein homolog)

Application Details

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

Restrictions: For Research Use only

Handling

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

References for anti-ANKH antibody (ABIN965554)

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).

Target Details for ANKH

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

Alternative Name: ANKH (ANKH Products)

Synonyms: 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

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.