ANKH anticorps (N-Term)

N° du produit ABIN965554

Cet anticorps Lapin Polyclonal détecte spécifiquement ANKH dans IHC. Il présente une réactivité envers Humain et Souris et a été mentionné dans 3+ publications.

Aperçu rapide pour ANKH anticorps (N-Term) (ABIN965554)

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

Reactivité: Humain, Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp ANKH est non-conjugé

Application: Immunohistochemistry (IHC)

Détail du produit anti-ANKH anticorps

Épitope: N-Term

Purification: Purified by antigen-specific affinity chromatography.

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

Information d'application

Indications d'application: ELISA, Western blotting: 1µg/ml for 2hrs.

Restrictions: For Research Use only

Stockage

Format: Liquid

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

Agent conservateur: Sodium azide

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

Stock: -20 °C

Références pour anticorps anti-ANKH (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." dans: 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." dans: 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." dans: Science (New York, N.Y.), Vol. 289, Issue 5477, pp. 265-70, (2000) (PubMed).

Détails sur ANKH

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

Autre désignation: ANKH

Sujet: 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.