Mouse ABCA4 (Rim Protein) Monoclonal Antibody | anti-ABCA4 antibody

Anti-ABCA4 (Rim Protein)

Cat. Num. AAA72744

• Gene Names: Abca4; RmP; Abcr; Abc10; AW050280; D430003I15Rik
• Reactivity: Bovine, human, mouse, Xenopus
• Applications: Immunohistochemistry, Western Blot
• Purity: Protein G purified (Protein G purified culture supernatant)
• Synonyms: ABCA4 (Rim Protein), Antibody; Anti-ABCA4 (Rim Protein); anti-ABCA4 antibody

• Host: Mouse
• Reactivity: Bovine, human, mouse, Xenopus
• Clonality: Monoclonal
• Isotype: IgG
• Clone Number: 3F4
• 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.
• Sequence Length: 2310

• Applicable Applications for anti-ABCA4 antibody: IHC (Immunohistochemistry), WB (Western Blot)
• Antigen: Partially purified bovine 220-kDa disc rim protein.
• Immunogen Information: Partially purified bovine 220-kDa disc rim protein
• Immunogen Species: Bovine
• 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 indentified 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.

Application Data

Related Product Information for anti-ABCA4 antibody

Mouse monoclonal antibody

References

• 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.

NCBI and Uniprot Product Information

• NCBI GI #: 60391189
• NCBI GeneID: 11304
• UniProt Accession #: O35600
• Molecular Weight: 220
• 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
• NCBI Official Synonym Symbols: RmP; Abcr; Abc10; AW050280; D430003I15Rik
• 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 Gene Name: Abca4
• UniProt Synonym Gene Names: Abcr; RIM protein; RmP
• UniProt Entry Name: ABCA4_MOUSE