Anti-Hamartin/TSC1 Antibody Picoband® Fluoro488 Conjugated

Hamartin also known as tuberous sclerosis 1 is a protein that in humans is encoded by the TSC1 gene. It is mapped to 9q34.13. This peripheral membrane protein was implicated as a tumor suppressor. It forms a complex with TSC2 that regulates mTORC1 signaling and may be also involved in vesicular transport and docking. Hamartin and TSC2 have critical roles in neuronal polarity, and that a common pathway regulates polarization and growth in neurons and cell size in other tissues. Hamartin is a growth inhibitory protein whose biologic effect is probably dependent on its interaction with tuberin. It also can affect cell proliferation via deregulation of G1 phase. Loss or perturbation of Hamartin function leads to loss of adhesion to the cellular matrix and initiates the development of TSC hamartomas.

E.coli-derived human Hamartin recombinant protein (Position: D686-Y884) . Human Hamartin shares 96% and 95% amino acid (aa) sequences identity with mouse and rat Hamartin, respectively.

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. .

1. Benvenuto, G., Li, S., Brown, S. J., Braverman, R., Vass, W. C., Cheadle, J. P., Halley, D. J. J., Sampson, J. R., Wienecke, R., DeClue, J. E. The tuberous sclerosis-1 (TSC1) gene product hamartin suppresses cell growth and augments the expression of the TSC2 product tuberin by inhibiting its ubiquitination. Oncogene 19: 6306-6316, 2000. 2. Choi, Y.-J., Di Nardo, A., Kramvis, I., Meikle, L., Kwiatkowski, D. J., Sahin, M., He, X. Tuberous sclerosis complex proteins control axon formation. Genes Dev. 22: 2485-2495, 2008. 3. Tapon, N., Ito, N., Dickson, B. J., Treisman, J. E., Hariharan, I. K. The Drosophila tuberous sclerosis complex gene homologs restrict cell growth and cell proliferation. Cell 105: 345-355, 2001.