ANKH Antikörper (N-Term)

Produktnummer ABIN965554

Produktdetails anti-ANKH Antikörper

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

Bindungsspezifität: N-Term

Reaktivität: Human, Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser ANKH Antikörper ist unkonjugiert

Applikation: Immunohistochemistry (IHC)

Aufreinigung: 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)

Anwendungsinformationen

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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: This antibody is stored in PBS, 50% glycerol

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Lagerung: -20 °C

Referenzen für anti-ANKH Antikörper (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).

Details zu ANKH

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

Andere Bezeichnung: ANKH (ANKH Produkte)

Synonyme: ANK antikoerper, CCAL2 antikoerper, CMDJ antikoerper, CPPDD antikoerper, HANK antikoerper, MANK antikoerper, Ank antikoerper, Ankh antikoerper, D15Ertd221e antikoerper, ank antikoerper, mKIAA1581 antikoerper, ankh antikoerper, wu:fc08d03 antikoerper, wu:fj64g09 antikoerper, zgc:110290 antikoerper, ANKH inorganic pyrophosphate transport regulator antikoerper, progressive ankylosis antikoerper, ANKH inorganic pyrophosphate transport regulator b antikoerper, ANKH inorganic pyrophosphate transport regulator S homeolog antikoerper, ANKH inorganic pyrophosphate transport regulator a antikoerper, ANKH antikoerper, Ankh antikoerper, Ank antikoerper, ankhb antikoerper, ankh.S antikoerper, ankha antikoerper

Hintergrund: 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.