Product Name
ABCA4 (Rim Protein), Monoclonal Antibody
Popular Item
Full Product Name
Anti-ABCA4 (Rim Protein)
Product Gene Name
anti-ABCA4 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Chromosome Location
Chromosome: 3; NC_000069.6 (122044035..122180070). Location: 3 G1; 3 52.94 cM
3D Structure
ModBase 3D Structure for O35600
Species Reactivity
Bovine, human, mouse, Xenopus
Specificity
Specific for ABCA4 protein in Western Blots of bovine, human, mouse and Xenopus tissues.
Purity/Purification
Protein G purified (Protein G purified culture supernatant)
Form/Format
100 ul in 10 mM HEPES (pH 7.5), 150 mM NaCl, 100 ug per ml BSA and 50% glycerol. Adequate amount of material to conduct 10-mini Western Blots.
Antigen
Partially purified bovine 220-kDa disc rim protein.
Immunogen Information
Partially purified bovine 220-kDa disc rim protein
Species Reactivity Note
The antibody has been directly tested for reactivity in bovine, human, mouse and Xenopus tissues.
Biological Significance
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).
Preparation and Storage
Store at -20 degree C in undiluted aliquots; stable for up to one year after date of receipt. Avoid freeze/thaw cycles.
Other Notes
Small volumes of anti-ABCA4 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-ABCA4 antibody
Mouse monoclonal antibody
Applications Tested/Suitable for anti-ABCA4 antibody
Western Blot (WB), Immunohistochemistry (IHC)
Application Notes for anti-ABCA4 antibody
Quality Control: Western blots performed on each lot.
WB: 1:1000
IHC: 1:100
Testing Data of anti-ABCA4 antibody
NCBI/Uniprot data below describe general gene information for ABCA4. It may not necessarily be applicable to this product.
NCBI Accession #
O35600.1
[Other Products]
UniProt Primary Accession #
O35600
[Other Products]
UniProt Related Accession #
O35600[Other Products]
NCBI Official Full Name
Retinal-specific ATP-binding cassette transporter
NCBI Official Synonym Full Names
ATP-binding cassette, sub-family A (ABC1), member 4
NCBI Official Symbol
Abca4 [Similar Products]
NCBI Official Synonym Symbols
RmP; Abcr; Abc10; AW050280; D430003I15Rik
[Similar Products]
NCBI Protein Information
retinal-specific ATP-binding cassette transporter; Rim protein; RIM ABC transporter; ATP-binding cassette 10; ATP-binding cassette sub-family A member 4
UniProt Protein Name
Retinal-specific ATP-binding cassette transporter
UniProt Synonym Protein Names
ATP-binding cassette sub-family A member 4; RIM ABC transporter
Protein Family
ABC transporter A family
UniProt Gene Name
Abca4 [Similar Products]
UniProt Synonym Gene Names
Abcr; RIM protein; RmP [Similar Products]
UniProt Entry Name
ABCA4_MOUSE
NCBI Summary for ABCA4
The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intracellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ABC1 subfamily. Members of the ABC1 subfamily comprise the only major ABC subfamily found exclusively in multicellular eukaryotes. This protein was the first of the ABC transporters to be observed in photoreceptors and may play a role in the photoresponse. Mutations in the human gene are found in patients diagnosed with Stargardt disease and are associated with retinitis pigmentosa-19 and macular degeneration age-related 2. [provided by RefSeq, Jul 2008]
UniProt Comments for ABCA4
Function: In the visual cycle, acts as an inward-directed retinoid flipase, retinoid substrates imported by ABCA4 from the extracellular or intradiscal (rod) membrane surfaces to the cytoplasmic membrane surface are all-trans-retinaldehyde (ATR) and N-retinyl-phosphatidyl-ethanolamine (NR-PE). Once transported to the cytoplasmic surface, ATR is reduced to vitamin A by trans-retinol dehydrogenase (tRDH) and then transferred to the retinal pigment epithelium (RPE) where it is converted to 11-cis-retinal. May play a role in photoresponse, removing ATR/NR-PE from the extracellular photoreceptor surfaces during bleach recovery
By similarity.
Subcellular location: Membrane; Multi-pass membrane protein
By similarity. Note: Localized to outer segment disk edges of rods and cones, with around one million copies/photoreceptor
By similarity.
Tissue specificity: Retinal-specific. Seems to be exclusively found in the rims of rod photoreceptor cells.
Disruption phenotype: Delayed dark adaptation but normal final rod threshold. Ref.3
Sequence similarities: Belongs to the ABC transporter superfamily. ABCA family.Contains 2 ABC transporter domains.
Product References and Citations for anti-ABCA4 antibody
• Michelle Illing, Laurie L. Molday and Robert S. Molday. The 220-kDa Rim Protein of Retinal Rod Outer Segments Is a Member of the ABC Transporter Superfamily. J. Biol. Chem., (1997) Vol 272 (15) I April 11. 10303-10310.
• Klevering BJ, Deutman AF, Maugeri A, Cremers FP, Hoyng CB (2005) The spectrum of retinal phenotypes casued by mutations in the ABCA4 gene. Graefes Arch Clin Exp Ophthalmol. 243(2):90-100.
• Molday, L. et al., ABCR expression in foveal cone photoreceptors and its role in Stargardt macular dystrophy. Nature Genetics (2000) 25, 257 - 258.
• 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.
Research Articles on ABCA4
1. Despite pronounced lipofuscin accumulation in the retinal pigment epithelium of Abca4(-/-) mice, ERG and histology showed a slow age-related thinning of the photoreceptor layer similar to wild type controls up to 12 months.
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