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品种反应性 主要应用 宿主 格式 抗体类型 H IHC, WB, ICC M Purified Monoclonal Antibody 描述 产品目录编号 MABN52 描述 Anti-REP-1 Antibody, clone 2F1 Alternate Names - TCD protein
- choroideremia (Rab escort protein 1)
- choroideremia
- Choroideraemia protein
- Rab escort protein 1
- Rab geranylgeranyltransferase component A
背景信息 Rab proteins are low molecular weight, ras-related GTPases that bind to a cell membrane’s cytoplasmic surface and function to regulate protein trafficking in both endocytic and secretory pathways. Rab escort protein (REP)-1 is a 653 amino acid protein belonging to the Rab GDI family of proteins. REP-1 binds to newly assembled unprenylated RAB proteins, escorts them to the Rab GGTases, and remains attached during and after the geranylgeranyl transfer reaction. REP proteins are composed of two conserved domains connected by a 150 amino acid insert. The multisheet domain I acts to assemble a Rab-binding platform. A globular, smaller, α-helical domain II, is thought to participate in membrane-protein interaction. Defects in the expression of REP-1 cause truncation or absence of the protein, and result in a disease called choroideremia (CHM), an X-linked blindness characterized by a progressive dystrophy of the retinal pigment epithelium, retina, and the choroid. 产品信息 格式 Purified 控制 - 293T cell lysate
演示 Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. 应用 应用 Anti-REP-1 Antibody, clone 2F1 is an antibody against REP-1 for use in WB, IP, IC, IH. 主要应用 - Immunohistochemistry
- Western Blotting
- Immunocytochemistry
应用说明 Immunohistochemistry Analysis: A previous lot of MABN52 was successfully used in IH, as reported by an independent laboratory (MacDonald, I.M., et al. (2005). Invest Ophthalmol Vis Sci. 46:E-Abstract 540). 生物信息 免疫原品种 Recombinant protein corresponding to human REP-1. 表位 Unknown 克隆 2F1 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 This antibody recognizes REP-1. 同种型 IgG1κ 品种反应性 Human Species Reactivity Note Demonstrated to react with human. 抗体类型 Monoclonal Antibody Entrez基因编号 - NP_000381
Entrez基因汇总 This gene encodes component A of the RAB geranylgeranyl transferase holoenzyme. In the dimeric holoenzyme, this subunit binds unprenylated Rab GTPases and then presents them to the catalytic Rab GGTase subunit for the geranylgeranyl transfer reaction. Rab GTPases need to be geranylgeranyled on either one or two cysteine residues in their C-terminus to localize to the correct intracellular membrane. Mutations in this gene are a cause of choroideremia; also known as tapetochoroidal dystrophy (TCD). This X-linked disease is characterized by progressive dystrophy of the choroid, retinal pigment epithelium and retina. Alternative splicing results in multiple transcript variants encoding different isoforms. 基因符号 - REP1
- CHM
- REP-1
- GGTA
- TCD
- HSD-32
纯化方法 Protein G purfied UniProt编号 - P24386
UniProt汇总 FUNCTION: Binds unprenylated Rab proteins, presents it to the catalytic Rab GGTase dimer, and remains bound to it after the geranylgeranyl transfer reaction. The component A is thought to be regenerated by transferring its prenylated Rab back to the donor membrane.
SUBUNIT STRUCTURE: Monomer. Interacts with RAB1B; the interaction is required for RAB1B prenylation. Interacts with RGGT (By similarity).
INVOLVEMENT IN DISEASE: Defects in CHM are the cause of choroideremia (CHM) [MIM:303100]; also known as tapetochoroidal dystrophy (TCD). CHM is a common form of X-linked blindness characterized by progressive dystrophy of the choroid, retinal pigment epithelium and retina.
SEQUENCE SIMLARITIES: Belongs to the Rab GDI family.产品使用声明 质量保证 Evaluated by Western Blot in 293T cell lysate.
Western Blot Analysis: 0.5 µg/mL of this antibody detected REP-1 on 10 µg of 293T cell lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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