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当前位置:首页 > 抗原抗体、ELISA、WB > 一抗 > Monoclonal Antibodies > ABCA4 (Rim Protein) Antibody

ABCA4 (Rim Protein) Antibody

货号 货期 规格 / 价格 询价
AMM05530G 100 μl / ¥5450

ABCA4 (Rim Protein) Antibody

品牌

Leading Biology

货号

AMM05530G

产品分类

Monoclonal Antibodies

研究领域

产品概述

We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format. We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team. This product is a high quality ABCA4 (Rim Protein) antibody.

分子量

220 KDa

宿主

Mouse

种属反应性

Bovine, Human, Mouse, Xenopus

靶点

Partially purified bovine 220-kDa disc rim protein.

克隆号

3F4

亚型

IgG

基因ID

UniProt ID

总结

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).

形式

Protein G purified culture supernatant

储存条件

Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

应用

IHC

稀释方法

IHC~~ 1:100

别名

Retinal-specific ATP-binding cassette transporter, ATP-binding cassette sub-family A member 4, RIM ABC transporter, RIM protein, RmP, Abca4, Abcr

图像

Immunohistochemical staining of adult mouse retina showingspecific immunolabeling of the ABCA4 protein. Photo courtesyof Mary Raven, University of California, Santa Barbara, CA.

说明书

数量

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