使用敲除细胞株进行验证RabMAb

Anti-Hamartin抗体[EP318Y] (ab40872)

概述

  • 产品名称Anti-Hamartin抗体[EP318Y]
    参阅全部 Hamartin 一抗
  • 描述
    兔单克隆抗体[EP318Y] to Hamartin
  • 经测试应用适用于: WB, IHC-P, Flow Cytmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide corresponding to C-terminal residues of human Hamartin.

  • 常规说明

    This product is a recombinant rabbit monoclonal antibody.

     

    Produced using Abcam’s RabMAb® technology. RabMAb® technology is covered by the following U.S. Patents, No. 5,675,063 and/or 7,429,487.

性能

应用

Our Abpromise guarantee covers the use of ab40872 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 1/20000. Predicted molecular weight: 150 kDa.
IHC-P Use at an assay dependent concentration.
Flow Cyt 1/1000 - 1/10000.

ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.

靶标

  • 功能In complex with TSC2, inhibits the nutrient-mediated or growth factor-stimulated phosphorylation of S6K1 and EIF4EBP1 by negatively regulating mTORC1 signaling. Seems not to be required for TSC2 GAP activity towards RHEB. Implicated as a tumor suppressor. Involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling.
  • 组织特异性Highly expressed in skeletal muscle, followed by heart, brain, placenta, pancreas, lung, liver and kidney. Also expressed in embryonic kidney cells.
  • 疾病相关Defects in TSC1 are the cause of tuberous sclerosis type 1 (TSC1) [MIM:191100]. It is an autosomal dominant multi-system disorder that affects especially the brain, kidneys, heart, and skin. TS1C is characterized by hamartomas (benign overgrowths predominantly of a cell or tissue type that occurs normally in the organ) and hamartias (developmental abnormalities of tissue combination). Clinical symptoms can range from benign hypopigmented macules of the skin to profound mental retardation with intractable seizures to premature death from a variety of disease-associated causes.
    Defects in TSC1 may be a cause of focal cortical dysplasia of Taylor balloon cell type (FCDBC) [MIM:607341]. FCDBC is a subtype of cortical displasias linked to chronic intractable epilepsy. Cortical dysplasias display a broad spectrum of structural changes, which appear to result from changes in proliferation, migration, differentiation, and apoptosis of neuronal precursors and neurons during cortical development.
  • 结构域The C-terminal putative coiled-coil domain is necessary for interaction with TSC2.
  • 翻译后修饰Phosphorylation at Ser-505 does not affect interaction with TSC2. Phosphorylated upon DNA damage, probably by ATM or ATR.
  • 细胞定位Cytoplasm. Membrane. At steady state found in association with membranes.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Hamartin antibody
    • kiaa0243 antibody
    • LAM antibody
    • TSC antibody
    • Tsc1 antibody
    • Tsc1 gene antibody
    • TSC1_HUMAN antibody
    • Tuberous sclerosis 1 antibody
    • Tuberous sclerosis 1 protein antibody
    • tumor suppressor antibody
    see all

Anti-Hamartin antibody [EP318Y] 图像



  • Predicted band size : 150 kDa

    Lane 1: Wild-type HAP1 cell lysate (20 µg)
    Lane 2: Hamartin knockout HAP1 cell lysate (20 µg)
    Lane 3: HeLa cell lysate (20 µg)
    Lane 4: Human skeletal muscle tissue lysate (20 µg)
    Lanes 1 - 4: Merged signal (red and green). Green - ab40872 observed at 150 kDa. Red - loading control, ab8245, observed at 37 kDa.
    ab40872 was shown to recognize Hamartin when Hamartin knockout samples were used, along with additional cross-reactive bands. Wild-type and Hamartin knockout samples were subjected to SDS-PAGE. ab40872 and ab8245 (loading control to GAPDH) were diluted 1/5000 and 1/10000 respectively and incubated overnight at 4°C. Blots were developed with goat anti-rabbit IgG (H + L) and goat anti-mouse IgG (H + L) secondary antibodies at 1/10000 dilution for 1 hour at room temperature before imaging.

  • Ab40872 staining human Hamartin in human kidneys by immunohistochemistry using paraffin embedded tissue.
  • Overlay histogram showing HeLa cells stained with ab40872 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab40872, 1/10000 dilution) for 30 min at 22°C. The secondary antibody used was Alexa Fluor® 488 goat anti-rabbit IgG (H&L) (ab150077) at 1/2000 dilution for 30 min at 22°C. Isotype control antibody (black line) was rabbit IgG (monoclonal) (0.1μg/1x106 cells) used under the same conditions. Unlabelled sample (blue line) was also used as a control. Acquisition of >5,000 events were collected using a 20mW Argon ion laser (488nm) and 525/30 bandpass filter. This antibody gave a positive signal in HeLa cells fixed with 4% paraformaldehyde (10 min)/permeabilized with 0.1% PBS-Tween for 20 min used under the same conditions.
  • Anti-Hamartin antibody [EP318Y] (ab40872) at 1/20000 dilution + HeLa cell lysate

    Predicted band size : 150 kDa
    Observed band size : 150 kDa

Anti-Hamartin antibody [EP318Y] (ab40872)参考文献

This product has been referenced in:
  • Platt FM  et al. Spectrum of phosphatidylinositol 3-kinase pathway gene alterations in bladder cancer. Clin Cancer Res 15:6008-17 (2009). Read more (PubMed: 19789314) »
  • Pymar LS  et al. Bladder tumour-derived somatic TSC1 missense mutations cause loss of function via distinct mechanisms. Hum Mol Genet 17:2006-17 (2008). Read more (PubMed: 18397877) »

See all 2 Publications for this product

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"