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ABCA4 (Rim Protein) Antibody

Catalog # Availability Size / Price Inquiry
AMM05530G 100 μl / $545

ABCA4 (Rim Protein) Antibody

Brand

Leading Biology

Catalog Number

AMM05530G

Product Type

Monoclonal Antibodies

Field of Research

Product Overview

We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format. We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team. This product is a high quality ABCA4 (Rim Protein) antibody.

Molecular Weight

220 KDa

Host

Mouse

Species Reactivity

Bovine, Human, Mouse, Xenopus

Target

Partially purified bovine 220-kDa disc rim protein.

Clone

3F4

Isotype

IgG

GeneID

UniProt ID

Summary

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).

Form

Protein G purified culture supernatant

Storage & Stability

Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

Applications

IHC

Dilution

IHC~~ 1:100

Synonyms

Retinal-specific ATP-binding cassette transporter, ATP-binding cassette sub-family A member 4, RIM ABC transporter, RIM protein, RmP, Abca4, Abcr

Images

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

Specification

Quantity

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