ABCA4 (Rim Protein) Antibody

Catalog #: 115-ABCA4 Categories: , , , Datasheet:

$109.00$365.00

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Retinal photoreceptor cell marker!

 

Formulation:
Protein G Purified
Species Tested:
Mouse, Human, Xenopus, Bovine
Applications:
WB 1:1000IHC 1:100
Species:
Mouse monoclonal
Gene Name:
ABCA4
Molecular Weight:
~220 kDa
Cite This Antibody:
PhosphoSolutions Cat# 115-ABCA4, RRID:AB_2492034
Antigen/Purification: ExpandCollapse

The antigen is a partially purified bovine ~220 kDa disc rim protein.

The antibody is Protein G purified culture supernatant.

Biological Significance: ExpandCollapse

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).

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

Storage

100 µl in 10 mM HEPES (pH 7.5), 150 mM NaCl, 100 µg per ml BSA and 50% glycerol. Adequate amount of material to conduct 10-mini Western Blots.

For long term storage –20° C is recommended in undiluted aliquots. Stable at –20° C for up to one year after date of receipt. Avoid freeze/thaw cycles.

Product Specific Protocols

Western Blotting

Click here to view our protocols page for Western blotting and lysate preparation.

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

  • 5 – Excellent (publishable, performed ideally)
  • 4 – Good (publishable, would use again)
  • 3 – Average (publishable, might use again)
  • 2 – Poor (unpublishable, signal inconclusive)
  • 1 – No signal (unpublishable)
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ABCA4 (Rim Protein) Antibody
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.

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