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.
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).
Protein G Purified
Monoclonal
3F4
IgG1
IHC, WB
Mouse
ABCA4
220 kDa
Partially purified bovine 220-kDa disc rim protein
Bovine, Human, Mouse, Xenopus
AB_2492034
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.
Liquid
Protein G purified culture supernatant.
10 mM HEPES (pH 7.5), 150 mM NaCl, 100 µg per ml BSA and 50% glycerol.
WB: 1:1000
WB Brain: 1:1000
IHC: 1:100
Unconjugated
Specific for endogenous levels of ABCA4.
Immunostaining performed on each lot.
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.
After date of receipt, stable for at least 1 year at -20°C.
Blue Ice
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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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