深圳欣博盛生物科技有限公司 a1

ABCA4 (aa1336-49) antibody, Internal
货号:GTX88465 规格:100μg 目录价:¥5000
产品详情
* 以下信息仅供参考,详情请以原厂网站为准
产品名称:
ABCA4 (aa1336-49) antibody, Internal
别名:
ATP binding cassette subfamily A member 4 , ABC10 , ABCR , ARMD2 , CORD3 , FFM , RMP , RP19 , STGD , STGD1
反应种属:
Human
宿主来源:
Goat
实验应用:
ELISA
同种型:
IgG
免疫原:
Peptide with sequence EPECPGPQLNTGTQ, from the internal region of the protein sequence according to NP_000341.2.
克隆性:
Polyclonal
克隆号:
纯化方式:
Purified by ammonium sulphate precipitation followed by antigen affinity chromatography
偶联:
Unconjugated
产品浓度:
0.50 mg/ml (Please refer to the vial label for the specific concentration.)
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
产品形式:
Liquid
存储溶液:
TBS, 0.5% BSA, 0.02% Sodium azide.
生产商:
GeneTex
功能与背景:
The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intracellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ABC1 subfamily. Members of the ABC1 subfamily comprise the only major ABC subfamily found exclusively in multicellular eukaryotes. 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 essental molecule across the photoreceptor cell membrane. Mutations in this gene are found in patients diagnosed with Stargardt disease, a form of juvenile-onset macular degeneration. Mutations in this gene are also associated with retinitis pigmentosa-19, cone-rod dystrophy type 3, early-onset severe retinal dystrophy, fundus flavimaculatus, and macular degeneration age-related 2. [provided by RefSeq, Jul 2008]
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