ABCA4 (Rim Protein) Antikörper

Antigen: ABCA4 (Rim Protein)

Klonalität: Monoklonal (3F4)

Wirt: Maus

Reaktivität: Rind (Kuh), Human, Maus, Xenopus laevis

Applikation: Western Blot (WB), Immunhistochemie (IHC)

Produktnummer: ABIN361347

Produktbeschreibung

Isotyp: IgG  (Passende Sekundärantikörper)

Klon: 3F4

Beschreibung: ABCA4 (ATP-binding cassette, sub-family A (ABC1), member 4, Rim Protein) is a member of the superfamily of ATP-binding cassette (ABC) transporters (Illing et al., 1997). ABC proteins transport various molecules across extra- and intracellular membranes. This protein is a retina-specific ABC transporter with N-retinylidene-PE as a substrate. It is expressed exclusively in retina photoreceptor cells, indicating the gene product mediates transport of an essential molecule across the photoreceptor cell membrane. Mutations in this gene are found in patients diagnosed with Stargardt disease and are associated with retinitis pigmentosa-19 and age-related macular degeneration (Wiszniewski et al., 2003). Defects in ABCA4 are the cause of Stargardt disease type 1 (STGD1) (Molday et al., 2000). STGD is one of the most frequent causes of macular degeneration in childhood. Defects in ABCA4 are also known to cause fundus flavimaculatus (FFM), age-related macular degeneration type 2 (ARMD2) and cone-rod dystrophy type 3 (CORD3) (Klevering et al., 2005). Anti-ABCA4  Immunohistochemical staining of adult mouse retina showing specific immunolabeling of the ABCA4 protein. Photo courtesy of Mary Raven, University of California, Santa Barbara, CA.

Spezifität: Specific for ABCA4

Anwendungen

Applikationshinweise: Recommended Dilution: WB: 1:1000 IHC: 1:100 Quality Control: Western blots performed on each lot.

Reinigung: Protein G purified culture supernatant

Lagerung: Store at -20 °C, stable for 1 year

Beschränkungen: Nur für Forschungszwecke einsetzbar

Bilder

Immunohistochemical staining of adult mouse retina showing specific immunolabeling of the ABCA4 protein. Photo courtesy of Mary Raven, University of California, Santa Barbara, CA.