Anti-ABCA4 Antibody, clone TMR1

产品说明

一般描述

Retinal-specific ATP-binding cassette transporter (UniProt F1MWM0/P78363; also known as ATP-binding cassette sub-family A member 4, ABC transporter A4, RIM ABC transporter, RIM protein, RmP, Stargardt disease protein) is encoded by the ABCA4 (also known as ABCR) gene (Gene ID 281584/24) in bovine/human species. ABC transporter A4, a ~250 kDa single-chain, is a multi-pass membrane protein of the superfamily of ATP-binding cassette (ABC) transporters that is localized to the disk margins of vertebrate photoreceptor outer segments. It is implicated in the clearance of all-trans retinal derivatives from rod and cone photoreceptor cells following photoexcitation as part of the visual cycle. ABC transporter A4 is composed of two symmetrically organized halves, each comprising six membrane-spanning helices, a large glycosylated exocytoplasmic domain located inside the disk, and a cytoplasmic domain with an ATP-binding cassette. Mutations in ABCA4 are known to cause Stargardt macular degeneration, which is an autosomal recessive form of juvenile macular degeneration associated with a severe loss in vision.

Ref.: Tsybovsky, Y et al. (2010). Adv. Exp. Med. Biol. 703, 105-125.

特异性

Clone TMR1 immunostained retina cryosections from wild-type mice, but not retina from mice with homozygous L541P;A1038V (PV) missense mutation (Courtesy of Grace Jin, Case Western University, U.S.A.).

免疫原

ABCA4 isolated from bovine retinas (Zhang, N., et al. (2015). Hum. Mol. Genet. 24(11):3220-3237).

应用

Immunofluorescence Analysis: A 1:25-125 dilution from a representative lot detected ABCA4 immunoreactivity in retina cryosections from wild-type mice, but not in retina from mice with homozygous L541P;A1038V (PV) missense mutation (Courtesy of Grace Jin, Case Western University, U.S.A.).

Immunocytochemistry Analysis: A representative lot detected similar cellular localization of wild-type and ABCA4 missense constructs with either or both L541P and A1038V mutation by fluorescent immunocytochemistry staining of 4% paraformaldehyde-fixed, 0.1% Triton X-100-permeabilized HEK293 transfectants (Zhang, N., et al. (2015). Hum. Mol. Genet. 24(11):3220-3237).

Immunofluorescence Analysis: A representative lot detected ABCA4 immunoreactivity in retina cryosections from wild-type mice and mice with heterozygous L541P;A1038V (PV) missense mutaion, but not mice with homozygous PV mutation (Zhang, N., et al. (2015). Hum. Mol. Genet. 24(11):3220-3237).

Research Category
Neuroscience
Detect ABCA4 using this mouse monoclonal Anti-ABCA4 Antibody, clone TMR1, Cat. No. MABN1775, validated for use in Immunocytochemistry, Immunofluorescence, and Western Blotting.

质量

Evaluated by Western Blotting in human retina tissue lysate.

Western Blotting Analysis: A 1:125 dilution of this antibody detected ABCA4 in 10 µg of human retina tissue lysate.

目标描述

~257 kDa observed. 257.4/255.9/260.2 kDa (bovine/human/mouse) calculated. Uncharacterized bands may be observed in some lysate(s).

外形

Protein G purified.
Format: Purified
Purified mouse IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

储存及稳定性

Stable for 1 year at 2-8°C from date of receipt.

其他说明

Concentration: Please refer to lot specific datasheet.

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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