全部產(chǎn)品分類
品牌 |
Leading Biology | 貨號 |
AMM05530G |
產(chǎn)品分類 |
Monoclonal Antibodies | 研究領(lǐng)域 |
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產(chǎn)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.
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分子量 |
220 KDa
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宿主 |
Mouse
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種屬反應(yīng)性 |
Bovine, Human, Mouse, Xenopus
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靶點 |
Partially purified bovine 220-kDa disc rim protein.
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克隆號 |
3F4
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亞型 |
IgG
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基因ID |
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UniProt ID |
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總結(jié) |
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).
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形式 |
Protein G purified culture supernatant
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儲存條件 |
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.
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應(yīng)用 |
IHC
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稀釋方法 |
IHC~~ 1:100
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別名 |
Retinal-specific ATP-binding cassette transporter, ATP-binding cassette sub-family A member 4, RIM ABC transporter, RIM protein, RmP, Abca4, Abcr
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圖像 |
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說明書 |
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數(shù)量 |
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