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Purified anti-Hamartin (TSC1) Antibody

Pricing & Availability
Clone
1B2-2A8 (See other available formats)
Regulatory Status
RUO
Other Names
Hamartin, Tuberous sclerosis 1 protein, KIAA0243, TSC, TSC 1
Isotype
Mouse IgG1, κ
Ave. Rating
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Product Citations
publications
1B2-2A8_PURE_Hamartin_TSC1_Antibody_041117
Total cell lysates (15 µg protein) from A431, HeLa and NIH3T3 were resolved by 4-12% Bis-Tris gel electrophoresis, transferred to nitrocellulose, and probed with 2 µg/mL purified anti-Hamartin (TSC1) (clone 1B2-2A8) antibody (upper). Proteins were visualized using a goat anti-mouse-IgG secondary antibody conjugated to HRP and chemiluminescence detection. Direct-Blot™ HRP anti-β-actin antibody was used as a loading control (lower). Lane M is the molecular weight ladder.
  • 1B2-2A8_PURE_Hamartin_TSC1_Antibody_041117
    Total cell lysates (15 µg protein) from A431, HeLa and NIH3T3 were resolved by 4-12% Bis-Tris gel electrophoresis, transferred to nitrocellulose, and probed with 2 µg/mL purified anti-Hamartin (TSC1) (clone 1B2-2A8) antibody (upper). Proteins were visualized using a goat anti-mouse-IgG secondary antibody conjugated to HRP and chemiluminescence detection. Direct-Blot™ HRP anti-β-actin antibody was used as a loading control (lower). Lane M is the molecular weight ladder.
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696502 100 µg $276
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Description

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder that is due to inactivating mutations in TSC1 (also known as hamartin) or TSC2 (also known as tuberin). TSC is featured by benign tumors (hamartomas) involving multiple organ systems. TSC1, TSC2 and TBC1 domain family member 7 (TBC1D7) form the TSC protein complex that acts as an inhibitor of the mechanistic target of rapamycin (mTOR) signaling, which in turn plays a critical role in regulating cell growth, proliferation, autophagy and protein and lipid synthesis. The TSC protein complex controls the activity of mTORC1 via RAS homologue enriched in brain (RHEB). mTORC1 downstream targets include phospho‑p70 ribosomal S6 kinase, phospho-ribosomal protein S6 and phospho‑4EBP1 (eukaryotic translation initiation factor 4E‑binding protein 1). Activation of mTORC1 enhances protein translation, and hyperactivation of mTORC1 has been discovered to lead to extensive metabolic reprogramming, including effects on glycolysis, autophagy, nucleotide biosynthesis and lipid biosynthesis. In many cases, this reprogramming leads to vulnerabilities that induce cell death indicating that the altered metabolism of cells with mTORC1 hyperactivation will provide therapeutic opportunities. By contrast, inhibition of mTORC1 with allosteric inhibitors, such as sirolimus and everolimus, restores TSC2‑deficient cells to metabolic homeostasis and may thereby ‘protect’ them from cell death.

In addition to the canonical TSC–RHEB–mTORC1 pathway, there is evidence of non-canonical pathways. These include targets of TSC1 that are TSC2 independent, targets of TSC1 and TSC2 that are RHEB independent and targets of RHEB that are mTORC1 independent.
 

Product Details
Technical data sheet

Product Details

Verified Reactivity
Human, Mouse
Antibody Type
Monoclonal
Host Species
Mouse
Immunogen
Partial human TSC1 recombinant protein (480-666 a.a.)
Formulation
Phosphate-buffered solution, pH 7.2, containing 0.09% sodium azide.
Preparation
The antibody was purified by affinity chromatography.
Concentration
0.5 mg/ml
Storage & Handling
The antibody solution should be stored undiluted between 2°C and 8°C.
Application

WB - Quality tested
Recommended Usage

Each lot of this antibody is quality control tested by Western blotting. For Western blotting, the suggested use of this reagent is 0.5 - 5.0 µg per ml. It is recommended that the reagent be titrated for optimal performance for each application.

Application Notes

As per the Human Protein Atlas RNA level dataset, A431 is expected to have a low level of TSC1 expression.

This clone weakly cross-reacts with mouse.

Clone 1B2-2A8 does not work for IF (in-house tested).

RRID
AB_2687066 (BioLegend Cat. No. 696502)

Antigen Details

Structure
1164 amino acids with a predicted molecular weight of 130 kD.
Distribution

Cytoplasm, Membrane

Function
In complex with TSC2, inhibits the nutrient-mediated or growth factor-stimulated phosphorylation of S6K1 and EIF4EBP1 by negatively regulating mTORC1 signaling.
Interaction
Interacts with TSC2, DOCK7, FBXW5 and TBC1D7.
Biology Area
Apoptosis/Tumor Suppressors/Cell Death, Cell Biology, Signal Transduction
Antigen References

1. Henske EP, et al. 2016. Nat. Rev. Dis. Primers. 2: 16035.
2. Kwiatkowski DJ and Manning BD. 2014. N. Engl. J. Med. 371: 778.
3. Dibble CC and Cantley LC. 2015, Trends Cell Biol. 25:545.
4. Neuman NA and Henske EP. 2011. EMBO Mol. Med. 3:189.
5. Han JM and Sahin M. 2011. FEBS Lett. 585:973.

Gene ID
7248 View all products for this Gene ID
UniProt
View information about Hamartin on UniProt.org

Related FAQs

There are no FAQs for this product.
Go To Top Version: 0    Revision Date: 04/14/2017

For Research Use Only. Not for diagnostic or therapeutic use.

 

This product is supplied subject to the terms and conditions, including the limited license, located at www.biolegend.com/terms) ("Terms") and may be used only as provided in the Terms. Without limiting the foregoing, BioLegend products may not be used for any Commercial Purpose as defined in the Terms, resold in any form, used in manufacturing, or reverse engineered, sequenced, or otherwise studied or used to learn its design or composition without express written approval of BioLegend. Regardless of the information given in this document, user is solely responsible for determining any license requirements necessary for user’s intended use and assumes all risk and liability arising from use of the product. BioLegend is not responsible for patent infringement or any other risks or liabilities whatsoever resulting from the use of its products.

 

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This data display is provided for general comparisons between formats.
Your actual data may vary due to variations in samples, target cells, instruments and their settings, staining conditions, and other factors.
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