antibody

Hamartin (TSC1, Tuberous Sclerosis 1)

H1779-01

100 ul

polyclonal

domestic rabbit

nonconjugated

Hamartin (TSC1, Tuberous Sclerosis 1)

rabbit

Pab

Antibodies

100 ul

IgG

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder that causes symptoms including hamartomas in brain, kidney, heart, lung and skin (1). The tumor suppressor genes TSC1 and TSC2 encode hamartin and tuberin, respectively (2, 3). Hamartin and tuberin form a functional complex and are involved in numerous cellular activities such as vesicular trafficking, regulation of the G1 phase of the cell cycle, steroid hormone regulation, Rho activation and anchoring neuronal intermediate filaments to the actin cytoskeleton (4–9). Cells lacking hamartin or tuberin display phosphorylation of S6 kinase and S6 resulting in negative regulation of S6 kinase. Furthermore, the combination of genetic, biochemical and cell-biological studies demonstrate that the tuberin:hamartin complex functions as a GTPase-activating protein for the Rasrelated G protein Rheb, and thus inhibits targets of rapamycin including mTOR (10). Hamartin is phosphorylated by CDK1 (cdc2) at Thr417, Ser584 and Thr1047 in cells in G2/M phase of the cell cycle (11). Applications: Suitable for use in Western Blot, Immunoprecipitation. Other applications not tested. Recommended Dilution: Western Blot: 1:1000 Immunoprecipitation: 1:50 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot. Store at -20°C. Aliquots are stable for at least 12 months at -20°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.

Hu Mo Rt

Synthetic peptide (KLH-coupled) corresponding to the sequence of human Hamartin.

Detects endogenous levels of total human Hamartin/TSC1 protein. Species Crossreactivity: mouse and rat

Protein A and peptide affinity chromatography.

Supplied as a liquid in 10mM sodium HEPES, pH 7.5, 150mM sodium chloride, 100ug/ml BSA, 50% glycerol.

Not determined

Product Type: Pab Isotype: IgG Host: rabbit Source: human Concentration: Not determined Form: Supplied as a liquid in 10mM sodium HEPES, pH 7.5, 150mM sodium chloride, 100ug/ml BSA, 50% glycerol. Purity: Protein A and peptide affinity chromatography. Immunogen: Synthetic peptide (KLH-coupled) corresponding to the sequence of human Hamartin. Specificity: Detects endogenous levels of total human Hamartin/TSC1 protein. Species Crossreactivity: mouse and rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.

IP WB

-20°C

(1) Sparagana, S.P. and Roach, E.S. (2000) Curr. Opin. Neurol. 13, 115–119. (2) van Slegtenhorst, M. et al. (1997) Science 277, 805–808. (3) No authors listed, (1993) Cell 75, 1305–1315. (4) Plank, T.L. et al. (1998) Cancer Res. 58, 4766–4770. (5) Xiao, G. et al. (1997) J. Biol. Chem. 272, 6097–6100. (6) Tapon, N. et al. (2001) Cell 105, 345–355. (7) Henry, K.W. et al. (1998) J. Biol. Chem. 273, 20535–20539. (8) Lamb, R.F. et al. (2000) Nat. Cell Biol. 2, 281–287. (9) Haddad, L.A. et al. (2002) J. Biol. Chem. 277, 44180–44186. (10) Manning, B.D. and Cantley, L.C. (2003) Trends Biochem Sci. 28, 573–576. (11) Astrinidis, A. et al. (2003) J. Biol. Chem. 278, 51372–51379.

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