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MABN52 Anti-REP-1 Antibody, clone 2F1

Anti-REP-1 Antibody, clone 2F1 is an antibody against REP-1 for use in WB, IP, IC, IH.

Anti-REP-1 Antibody, clone 2F1: のSDS（安全データシート）、CoA（試験成績書）およびCoQ（品質証明書）、カタログなど製品関連の技術資料を入手いただけます。
Research Category: Neuroscience Research Sub-Category: Neurodegenerative Diseases Gene Symbol: CHM, REP-1, GGTA, TCD, HSD-32, REP1 UniProt Number: P24386
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MABN52
100 µg  
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      概要

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      主要スペック表

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HWB, IP, ICC, IHCMPurifiedMonoclonal Antibody
      Description
      Catalogue NumberMABN52
      DescriptionAnti-REP-1 Antibody, clone 2F1
      Alternate Names
      • choroideremia (Rab escort protein 1)
      • choroideremia
      • Choroideraemia protein
      • Rab escort protein 1
      • Rab geranylgeranyltransferase component A
      • TCD protein
      Background InformationRab 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.
      References
      Product Information
      FormatPurified
      Control
      • 293T cell lysate
      PresentationPurified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationAnti-REP-1 Antibody, clone 2F1 is an antibody against REP-1 for use in WB, IP, IC, IH.
      Key Applications
      • Western Blotting
      • Immunoprecipitation
      • Immunocytochemistry
      • Immunohistochemistry
      Application NotesImmunohistochemistry 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).
      Biological Information
      ImmunogenRecombinant protein corresponding to human REP-1.
      EpitopeUnknown
      Clone2F1
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostMouse
      SpecificityThis antibody recognizes REP-1.
      IsotypeIgG1κ
      Species Reactivity
      • Human
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThis 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.
      Gene Symbol
      • CHM
      • REP-1
      • GGTA
      • TCD
      • HSD-32
      • REP1
      Purification MethodProtein G Purified
      UniProt Number
      UniProt SummaryFUNCTION: 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.
      Molecular Weight~83 kDa observed
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated 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.
      Usage Statement
      • 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.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      カタログ番号 GTIN
      MABN52 04053252517365

      Documentation

      Anti-REP-1 Antibody, clone 2F1 (M)SDS

      タイトル

      英語版製品安全データシート（(M)SDS） 

      Anti-REP-1 Antibody, clone 2F1 試験成績書（CoA）

      タイトルロット番号
      Anti-REP-1, clone 2F1 2483254
      Anti-REP-1, clone 2F1 - 2391170 2391170
      Anti-REP-1, clone 2F1 - 2444683 2444683
      Anti-REP-1, clone 2F1 - 1994921 1994921
      Anti-REP-1, clone 2F1 - 2971252 2971252
      Anti-REP-1, clone 2F1 - 3069694 3069694
      Anti-REP-1, clone 2F1 - 3270757 3270757
      Anti-REP-1, clone 2F1 - 3318358 3318358
      Anti-REP-1, clone 2F1 - 3465955 3465955
      Anti-REP-1, clone 2F1 - 3901385 3901385
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