Immunohistochemical staining of adult mouse retina showing specific immunolabeling of the ABCA4 protein (red, 1;100). Photo courtesy of Mary Raven, University of California, Santa Barbara, CA.

Immunohistochemical staining of adult mouse retina showing specific immunolabeling of the ABCA4 protein (red, 1;100). Photo courtesy of Mary Raven, University of California, Santa Barbara, CA.

Anti-ABCA4 (Rim Protein) (115-ABCA4)

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Product Details


Details

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).

Volume: 100 µL

Concentration: Lot Specific

Clonality: Monoclonal

Form: Protein G purified culture supernatant

Host Species: Mouse

Antibody Isotype: IgG

Species Reactivity: Bovine, Human, Mouse, Xenopus

Applications: WB, IHC

Immunogen: Partially purified bovine 220-kDa disc rim protein

Gene ID: ABCA4

Antibody Registry ID (RRID): AB_2492034

Physical State: Liquid

Conjugate: Unconjugated

Buffer: 100 µl in 10 mM HEPES (pH 7.5), 150 mM NaCl, 100 µg per ml BSA and 50% glycerol.

Validation and Application Notes

Molecular Weight: 220

Storage

-20 C

NOTE

Aves Labs products are intended for use as research laboratory reagents. They are not intended for use as diagnostic or therapeutic reagents in humans.


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