ABCA4 抗体
Our Local Distributor
北京 101111
Quick Overview for ABCA4 抗体 (ABIN371821)
抗原
See all ABCA4 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
-
-
特异性
- This antibody reacts with ABCA4 (Rim Protein).
-
交叉反应 (详细)
- Species reactivity (tested):Bovine, Human, Mouse and Xenopus.
-
产品特性
- Synonyms: ABCR, Stargardt disease protein, ATP-binding cassette sub-family A member 4,Retinal-specific ATP-binding cassette transporter
-
纯化方法
- Protein G Chromatography.
-
免疫原
- Partially purified bovine 220-kDa disc rim protein.
-
亚型
- IgG
-
-
-
-
应用备注
-
Immunohistochemistry: 1/100. Western blotting: 1/1000.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user. -
限制
- 仅限研究用
-
-
-
状态
- Liquid
-
缓冲液
- 10 mM HEPES ( pH 7.5) 150 mM NaCl, 100 μg/mL BSA and 50 % Glycerol.
-
储存条件
- -20 °C
-
储存方法
-
Store the antibody undiluted (in aliquots) at -20 °C. Avoid repeated freezing and thawing.
Shelf life: one year from despatch. -
有效期
- 12 months
-
-
- ABCA4 (ATP-Binding Cassette, Sub-Family A (ABC1), Member 4 (ABCA4))
-
别名
- ABCA4
-
背景
- 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).Synonyms: ABCR, ATP-binding cassette sub-family A member 4, Retinal-specific ATP-binding cassette transporter, Stargardt disease protein
-
基因ID
- 24
-
UniProt
- P78363
抗原
-