概述

  • 产品名称Anti-Progerin抗体[13A4]
    参阅全部 Progerin 一抗
  • 描述
    小鼠单克隆抗体[13A4] to Progerin
  • 特异性This antibody specifically recognizes Progerin (LaminAdelta50) and does not cross-react with endogenous prelamin A.
  • 经测试应用适用于: WB, ICC/IF, ELISA, IPmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide corresponding to Human Progerin (C terminal) conjugated to Keyhole Limpet Haemocyanin (KLH).

  • 阳性对照
    • HeLa Flag-Progerin or primary progeria fibroblasts.
  • 常规说明

    This antibody is available as an azide-free product (see ab180465).

     

    Progerin is expressed as the result of a de novo point mutation in the lamin A gene and is the underlying cause of Hutchison-Gilford progeria syndrome (accelerated aging). There is now also evidence that small amounts of Progerin are also produced in normal cells and that this might have a link with the normal aging process (Cao et al., PNAS, 2007 Mar 20;104(12):4949-54.). Gly608Gly is the most frequent HGPS-associated mutation. It is a silent base substitution that activates a cryptic splice donor in exon 11 of LMNA (BOX 3). Use of this anomalous splice donor leads to the loss of 150 nucleotides from the 3' end of exon 11 in the mature lamin A mRNA, and internal deletion of 50 amino-acid residues from the C terminus of lamin A. Progerin is the resulting mutant protein. Progerin retains its C-terminal CAAX motif, and therefore is farnesylated.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
  • 存储溶液Preservative: 0.02% Sodium Azide
    Constituents: PBS, pH 7.4
  • Concentration information loading...
  • 纯度IgG fraction
  • Primary antibody说明Progerin is expressed as the result of a de novo point mutation in the lamin A gene and is the underlying cause of Hutchison-Gilford progeria syndrome (accelerated aging). There is now also evidence that small amounts of Progerin are also produced in normal cells and that this might have a link with the normal aging process (Cao et al., PNAS, 2007 Mar 20;104(12):4949-54.). Gly608Gly is the most frequent HGPS-associated mutation. It is a silent base substitution that activates a cryptic splice donor in exon 11 of LMNA (BOX 3). Use of this anomalous splice donor leads to the loss of 150 nucleotides from the 3' end of exon 11 in the mature lamin A mRNA, and internal deletion of 50 amino-acid residues from the C terminus of lamin A. Progerin is the resulting mutant protein. Progerin retains its C-terminal CAAX motif, and therefore is farnesylated.
  • 克隆单克隆
  • 克隆编号13A4
  • 骨髓瘤x63-Ag8.653
  • 同种型IgG1
  • 轻链类型kappa
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab66587 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB 1/1000. Detects a band of approximately 70 kDa.
ICC/IF 1/10.
ELISA Use at an assay dependent dilution.
IP Use at an assay dependent dilution. use 20ul/assay

靶标

  • 相关性Progerin is a 614 amino acid protein involved in Hutchinson-Gilford progeria syndrome. Progerin is most often generated by a point mutation (C1824T) in the LMNA gene that codes for lamin A and C. This mutation activates a cryptic splice site and gives rise to a form of lamin A with a 50-amino acids internal deletion within the carboxyl-terminal domain of the protein. Approximately 80% of Hutchinson-Gilford progeria syndrome cases carry a single copy of the most common mutation, a silent point mutation, G608G (GGC > GGT), within exon 11 of LMNA gene.
  • 细胞定位Cell Membrane, Cytoplasmic, intermediate filament and Nuclear
  • 数据库链接
  • 别名
    • 70 kDa lamin antibody
    • CDCD1 antibody
    • CDDC antibody
    • CMD1A antibody
    • CMT2B1 antibody
    • EMD2 antibody
    • FPL antibody
    • FPLD antibody
    • HGPS antibody
    • IDC antibody
    • LA delta 50 antibody
    • Lamin A delta 50 antibody
    • LDP1 antibody
    • LFP antibody
    • LGMD1B antibody
    • LMN1 antibody
    • LMNA antibody
    • LMNC antibody
    • PRO1 antibody
    • renal carcinoma antigen NYREN32 antibody
    see all

Anti-Progerin antibody [13A4] 图像

  • Ab66587 specifically detecting human Progerin by Western blotting.

    Western blot analysis (10% PAGE) of whole cell lysate of HeLa cells ectopically expressing Flag-tagged human proteins (protein accession number AAR29466). The membrane was cut into strips and each strip was incubated separately with the following antibodies: Lane 1: anti Lamin A/C antibody (1:2000), Lane 2: anti Flag-tag antibody (1:5000), Lane 3: ab66587 (1:500).
  • Lane 1 : Anti Lamin A/C 3A6-4C11 at 1/1000 dilution
    Lanes 2 & 5 : Anti-Progerin antibody [13A4] (ab66587) at 1/200 dilution
    Lanes 3 & 6 : Anti-Progerin antibody [13A4] (ab66587) at 1/500 dilution
    Lanes 4 & 7 : Anti-Progerin antibody [13A4] (ab66587) at 1/1000 dilution

    Lane 1 : HeLa cells stably expressing Flag-tagged human Progerin
    Lane 2 : HeLa cells stably expressing Flag-tagged human Progerin
    Lane 3 : HeLa cells stably expressing Flag-tagged human Progerin
    Lane 4 : HeLa cells stably expressing Flag-tagged human Progerin
    Lane 5 : HeLa cells stably expressing Flag-tagged human Progerin
    Lane 6 : HeLa cells stably expressing Flag-tagged human Progerin
    Lane 7 : HeLa cells stably expressing Flag-tagged human Progerin


    Observed band size : 70 kDa (why is the actual band size different from the predicted?)


    Exposure time : 2 minutes

Anti-Progerin antibody [13A4] (ab66587)参考文献

This product has been referenced in:

See all 4 Publications for this product

Product Wall

Je suisque vous avez eu des problèmes en coIP.Merci pour votre appel.

Voici le lien promis pour nos protocoles en ligne:
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Thank you for kindly confirming these details. I am sorry this vial of antibody has not worked for this customer.

As requested, we are pleased to arrange a free of charge replacement. I can confirm that 1 vial of ab66587 has been added to yo...

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Thank you for taking the time to complete our questionnaire and contact us. I am sorry to hear you have had difficulty obtaining satisfactory results from this antibody.

The details you have kindly provided will enable us to investigate this...

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Thank you for confirming these details and for your cooperation. The details provided enable us to closely monitor the quality of our products. I am sorry ab66587 did not perform as stated on the datasheet and for the inconvenience this has caused. As ...

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Thank you very much again for your reply. I appreciate the time you have spent in the laboratory and understand your concerns. As it appears to me your biotin-labeled secondary and the appropriate detection system are indeed working and it is regret...

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Thank you very much for confirming these details. Before proceeding with this case, I would appreciate if you could confirm which detection system was used with the biotin-labeled secondary antibody ab6788: Have you used a HRP-conjugated anti-biotin...

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Thank you for contacting us. I am sorry to hear you have been experiencing problems with one of our antibodies. The quality of our products is important to us and I would like to reassure you that we investigate all customer complaints. I would like to...

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Thank you for contacting us with your question about ab66587. I do not know the full history of the clones 13A4 and 13A4D4, however it is likely that one is a "subclone" of the other, meaning they are similar. Unfortunately we are not able to provide ...

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Thank you for your inquiry. We do have a human progerin protein in the catalogue as ab93918 that can be used as a positive control in WB. http://www.abcam.com/Progerin-protein-Human-ab93918.html I hope this information helps.  Please contac...

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