Hamartin, CT (TSC1, Tuberous Sclerosis 1)

Mutation within the TSC1 gene is responsible for Tuberous sclerosis (TSC), an autosomal dominant genetic disorder associated with the development of benign tumors (hamartomas), a condition also induced by mutation of the Tumour sclerosis complex 2 (TSC2) gene. \nMany proteins have been shown to play a role in the regulation of the serine/threonine kinase known as target of rapamycin (TOR), a central component in a complex signaling pathway which controls cell proliferation and cell cycle progression. Negative regulation of TOR activity occurs following the over-expression of the TSC1 and TSC2 gene products, hamartin and tuberin. These act by suppressing the phosphorylation of eukaryotic translation initiation factor 4E binding protein 1 (4EBP1) and ribosomal protein S6 kinase (S6K), downstream targets of TOR involved in mRNA translation. Furthermore the TOR activator protein Rheb has been identified as a target of TSC1-TSC2 complexes. \nTSC1 is highly expressed in a range of tissues including skeletal muscle, heart, brain, placenta, pancreas, lung, liver and the kidney. \n\nApplications: \nSuitable for use in Western Blot. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 2ug/ml\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20 degrees C. Aliquots are stable for at least 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

H1779-01H

Size

100ug

Applications

WB

Hosts

Rabbit

Reactivities

Hum, Mouse

Form

Supplied as a liquid in PBS, 0.02% sodium azide.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

References

1. Shamji, A.F., et al (2003) Integration of growth factor and nutrient signaling: implications for cancer biology. Mol. Cell. 12:271-80. 2. Inoki, K., et al (2005) Signaling by target of rapamycin proteins in cell growth control. Microbiol. Mol. Biol. Rev. 69:79-100. 3. Tabancay, A.P., et al (2003) Identification of dominant negative mutants of Rheb GTPase and their use to implicate the involvement of human Rheb in the activation of p70S6K. J. Biol. Chem. 278:39921-30. 4. Inoki, K,. et al (2003) Rheb GTPase is a direct target of TSC2 GAP activity and regulates mTOR signaling. Genes Dev. 17:1829-34.

Additional Info

Recognizes human Tumor Sclerosis 1 protein (TSC1), also known as Harmartin, a 130kD tumour suppressor protein. Species Crossreactivity: mouse.