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
抗原
ABCA4
(ATP-Binding Cassette, Sub-Family A (ABC1), Member 4 (ABCA4))
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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