Hamartin (TSC1, Tuberous Sclerosis 1, KIAA0243, TSC)

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.\n\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.\n\nApplications: \nSuitable for use in Western Blot. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1ug/ml\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

H1779-01D

Size

100ug

Applications

WB

Hosts

Rabbit

Reactivities

Hum, Mouse, Rat

Form

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

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

References

1. Inoki, K. et al. (2005) Signaling by target of rapamycin proteins in cell growth control. Microbiol. and Mol. Biol. Rev. 69: 79-100. 2. Tee, A.R. et al. (2002) Tuberous sclerosis complex-1 and -2 gene products function together to inhibit mammalian target of rapamycin (mTOR)-mediated downstream signaling. P.N.A.S 99: 13571-13576.

Additional Info

Recognizes an epitope within the internal region of the 130kD tumor suppressor protein, Tumor sclerosis complex 1 (TSC1). Species Crossreactivity: Rat, Mouse.