Hamartin/TSC1 (1B2) Mouse mAb #4963
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Product Name
- WB
Inquiry Info. # 4963
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Supporting Data
| REACTIVITY | H M R |
| SENSITIVITY | Endogenous |
| MW (kDa) | 150 to 170 |
| Source/Isotype | Mouse IgG1 |
Application Key:
- WB-Western Blotting
Species Cross-Reactivity Key:
- H-Human
- M-Mouse
- R-Rat
Product Information
Product Usage Information
| Application | Dilution |
|---|---|
| Western Blotting | 1:1000 |
Storage
Protocol
Specificity / Sensitivity
Species Reactivity:
Source / Purification
Monoclonal antibody is produced by immunizing animals 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). 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. Cells lacking hamartin or tuberin fail to inhibit phosphorylation of S6 kinase resulting in the activation of S6 ribosomal protein's translation of 5'TOP mRNA transcripts (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.
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