Product Pathways - Akt Signaling
Hamartin/TSC1 (1B2) Mouse mAb #4963
| Applications | Reactivity | Sensitivity | MW (kDa) | Source | Isotype |
|---|---|---|---|---|---|
| W | H M R | Endogenous | 150 to 170 | Mouse | IgG1 |
Applications Key:
W=Western Blotting
Reactivity Key:
H=Human
M=Mouse
R=Rat
Species enclosed in parentheses are predicted to react based on 100% sequence homology. Species cross-reactivity is determined by Western blot.
Specificity / Sensitivity
Hamartin/TSC1 (1B2) Mouse mAb detects endogenous levels of total hamartin protein.
Source / Purification
Monoclonal antibody is produced by immunizing mice with a recombinant protein from the central region of human harmartin.
Background
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 Ras-related small 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).
- Sparagana, S.P. and Roach, E.S. (2000) Curr. Opin. Neurol. 13, 115-119.
- van Slegtenhorst, M. et al. (1997) Science 277, 805-808.
- No authors listed. (1993) Cell 75, 1305-1315.
- Plank, T.L. et al. (1998) Cancer Res. 58, 4766-4770.
- Xiao, G. et al. (1997) J. Biol. Chem. 272, 6097-6100.
- Tapon, N. et al. (2001) Cell 105, 345-355.
- Henry, K.W. et al. (1998) J. Biol. Chem. 273, 20535-20539.
- Lamb, R.F. et al. (2000) Nat. Cell Biol. 2, 281-287.
- Haddad, L.A. et al. (2002) J. Biol. Chem. 277, 44180-44186.
- Manning, B.D. and Cantley, L.C. (2003) Trends Biochem Sci. 28, 573-576.
- Astrinidis, A. et al. (2003) J. Biol. Chem. 278, 51372-51379.
Application References
- Murthy, V. et al. (2000) Similarities and differences in the subcellular localization of hamartin and tuberin in the kidney. Am J Physiol Renal Physiol. 278, 737-746. This article references the use of Hamartin/TSC1 (1B2) Mouse mAb in the following applications: IF-P IP Western Blotting
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This product is for in vitro research use only and is not intended for use in humans or animals. This product is not intended for use as therapeutic or in diagnostic procedures.