Anti-ABCA4 (Rim Protein) Antibody (3F4)

Name: Anti-ABCA4 (Rim Protein) Antibody
Brand: PhosphoSolutions
SKU: 115-ABCA4
Price: 456.0 USD
Availability: InStock

Our Anti-ABCA4 (Rim Protein) primary antibody from PhosphoSolutions is mouse monoclonal. It detects bovine, human, mouse, and Xenopus ABCA4 (Rim Protein) and is Protein G purified. It is great for use in WB, IHC.

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.

Western blot of 70 day old mouse retina lysate showing immunolabeling of the ~220 kDa ABCA4 protein at a dilution of 1:1000.

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.

Click on image to zoom

Species Reactivity

Bovine, Human, Mouse, Xenopus

Applications

ELISA, IHC, WB

Host Species

Mouse

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Data Sheet

Trial Size

SKU: 115-ABCA4

Volume: 100 µL

Variant

Price:

$456.00

Quantity:

Ships: 1-2 business days

Product Details

Target

ABCA4 (Rim Protein)

Scientific Background

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).

Format

Protein G Purified

Clonality

Monoclonal

Clone

3F4

Isotype

IgG1

Applications

ELISA, IHC, WB

Host Species

Mouse

Gene Name

ABCA4

Molecular Weight

220 kDa

Immunogen

Partially purified bovine 220-kDa disc rim protein

Immunogen Species

Bovine

Species Reactivity

Bovine, Human, Mouse, Xenopus

Antibody Registry ID

AB_2492034

Storage

Storage at -20°C is recommended, as aliquots may be taken without freeze/thawing due to presence of 50% glycerol. Stable for at least 1 year at -20°C.

Physical State

Liquid

Production Notes

Protein G purified culture supernatant.

Buffer

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

Dilution Ranges

WB: 1:1000
WB Brain: 1:1000
IHC: 1:100

Conjugate

Unconjugated

Specificity

Specific for endogenous levels of ABCA4.

Quality Control

Immunostaining performed on each lot.

Usage Statement

For research use only. Not intended for therapeutic or diagnostic use. Use of all products is subject to our terms and conditions, which can be viewed on our website.

Expiration

After date of receipt, stable for at least 1 year at -20°C.

Synonyms

ABC 10; ABC A4; ABC1 member 4; ABC transporter retinal-specific; ABC1 member4; ABC10; ABCA 4; abcA4; ABCA4_HUMAN; ABCR; ARMD 2; ARMD2; ATP binding cassette 10; ATP binding cassette sub family A member 4; ATP binding cassette sub family A member4; ATP binding cassette transporter; ATP binding cassette transporter retinal specific; ATP binding cassette; sub family A (ABC1 member 4; ATP binding cassette sub family A (ABC1 member4; ATP binding cassette10; ATP binding transporter retina specific; ATP-binding cassette sub-family A member 4; CORD 3; CORD3; DKFZp781N1972; FFM; FLJ17534; Photoreceptor rim protein; Retina specific ABC transporter; Retinal specific ATP binding cassette transporter; Retinal-specific ATP-binding cassette transporter; RIM ABC transporter; RIM protein; RmP; RP 19; RP19; Stargardt disease protein; STGD; STGD1; ATP binding cassette sub family A; sub family A

UniProt ID

F1MWM0
UniProt Summary: Flippase that catalyzes in an ATP-dependent manner the transport of retinal-phosphatidylethanolamine conjugates like 11-cis and all-trans isomers of N-retinylidene-phosphatidylethanolamine (N-Ret-PE) from the lumen to the cytoplasmic leaflet of photoreceptor outer segment disk membranes, where 11-cis-retinylidene-phosphatidylethanolamine is then isomerized to its all-trans isomer and reduced by RDH8 to produce all-trans-retinol. This transport activity ensures that all-trans-retinal generated from photoexcitation and 11-cis-retinal not needed for the regeneration of rhodopsin and cone opsins are effectively cleared from the photoreceptors, therefore preventing their accumulation and the formation of toxic bisretinoid (PubMed:10075733, PubMed:20404325, PubMed:22735453, PubMed:23144455, PubMed:24097981, PubMed:29847635, PubMed:33375396). Displays ATPase activity in vitro in absence of retinal substrate (PubMed:33605212, PubMed:39128720, PubMed:29847635, PubMed:33375396). May display GTPase activity that is strongly influenced by the lipid environment and the presence of retinoid compounds (PubMed:22735453). Binds the unprotonated form of N-retinylidene-phosphatidylethanolamine with high affinity in the absence of ATP, and ATP binding and hydrolysis induce a protein conformational change that causes N-retinylidene-phosphatidylethanolamine release (By similarity). {ECO:0000250|UniProtKB:F1MWM0, ECO:0000269|PubMed:10075733, ECO:0000269|PubMed:20404325, ECO:0000269|PubMed:22735453, ECO:0000269|PubMed:23144455, ECO:0000269|PubMed:24097981, ECO:0000269|PubMed:29847635, ECO:0000269|PubMed:33375396, ECO:0000269|PubMed:33605212, ECO:0000269|PubMed:39128720}.

NCBI Gene ID

281584

Shipping

Blue Ice

Western Blot Protocol: Download

General References

Illing, M., Molday, L.L. and Molday, R.S., 1997. The 220-kDa rim protein of retinal rod outer segments is a member of the ABC transporter superfamily. Journal of Biological Chemistry, 272(15), pp.10303-10310. PMID: 9092582
Klevering BJ, Deutman AF, Maugeri A, Cremers FP, Hoyng CB (2005) The spectrum of retinal phenotypes caused by mutations in the ABCA4 gene. Graefes Arch Clin Exp Ophthalmol. 243(2):90-100. PMID: 15614537
Molday, L. et al., ABCR expression in foveal cone photoreceptors and its role in Stargardt macular dystrophy. Nature Genetics (2000) 25, 257 - 258. PMID: 10888868
Wiszniewski, W. et al., ABCA4 mutations causing mislocalization are found frequently in patients with severe retinal dystrophies. Human Molecular Genetics (2005) 14(19):2769-2778. PMID: 16103129

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Anti-ABCA4 (Rim Protein) Antibody (3F4)

SKU: 115-ABCA4

Product Details

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