|Application ||WB, IHC|
|Calculated MW||129767 Da|
|Other Names||Hamartin, Tuberous sclerosis 1 protein, TSC1, KIAA0243, TSC|
|Target/Specificity||A synthetic peptide corresponding to C-terminal residues of human TSC1/Hamartin was used as an immunogen.|
|Format||50 mM Tris-Glycine (pH 7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||TSC1/Hamartin Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||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.|
|Cellular Location||Cytoplasm. Membrane; Peripheral membrane protein. Note=At steady state found in association with membranes|
|Tissue Location||Highly expressed in skeletal muscle, followed by heart, brain, placenta, pancreas, lung, liver and kidney. Also expressed in embryonic kidney cells|
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Provided below are standard protocols that you may find useful for product applications.
Tuberous sclerosis complexes-1 (TSC1 also known as Hamartin) is a tumor suppressor that form a complex with TSC2 (Tuberin). This complex is known to control various cellular functions including cell cycle, endocytosis, adhesion, and transcription (1). The TSC1/TSC2 complex inhibits phosphorylation of S6kinase and 4E-BP1 through inactivation of mTOR (2). Recently it has been shown that TSC1/Hamartin is localized to the centrosome and negatively regulates Plk1 implicating TSC1/hamartin to the regulation of centrosome duplication (3). Tuberous sclerosis (TSC), an autosomal dominant disorder that affects 1 in 6000 individuals, is caused by a mutation in either the TSC1 or TSC2 tumor suppressor gene.
1. Lamb,et al. Nature Cell Biol 2:281-287
2. Manning BD, et al. Molec. Cell 10:151-162, 2002.
3. Astrinidis A, Senapedis W, et al. Hum Mol Genet 15:287-297, 2005.
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