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Hamartin/TSC1 Antibody
SPA-11471
| Size | Price |
| 100 µL | Online Inquiry |
| More Options | Online Inquiry |
| Target Information | |
|---|---|
| Target Name | TSC1 |
| Gene Abbr. | TSC1 |
| Gene ID | 7248 |
| Full Name | TSC complex subunit 1 |
| Alias | LAM, TSC |
| Introduction | Tuberous sclerosis complex (TSC) is an autosomal dominant disorder that causes symptoms including hamartomas in brain, kidney, heart, lung and skin. The tumor suppressor genes TSC1 and TSC2 encode hamartin and tuberin, respectively. 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. 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. Hamartin is phosphorylated by CDK1 (cdc2) at Thr417, Ser584 and Thr1047 in cells in G2/M phase of the cell cycle. |
| Product Details | |
|---|---|
| Host | Mouse |
| Clonality | Monoclonal |
| Clone No. | 488915 |
| Isotype | IgG2B |
| Immunogen | E. coli-derived recombinant human TSC1. Asp156-Thr300. Q92574. |
| Usage | |
|---|---|
| Application | WB, FC |
| Dilutions | Western Blot (1 µg/mL); Flow Cytometry (2.5 µg/10^6 cells) |
| MW(KDa) | 150-170 |
| Reactivity | Human |
| Specificity | Detects human TSC1 in direct ELISAs and Western Blots. In direct ELISAs, no cross-reactivity with recombinant human (rh) TSC2 (aa 550-850), rhTSC2 (aa 1506-1748), or recombinant mouse TSC22 (aa 1-143) is observed. |
| Storage & Handling | |
|---|---|
| Storage Buffer | Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. |
| Preservative | No Preservative |
| Storage Temp. | 12 months from date of receipt, -20 to -70 °C as supplied. 1 month, 2 to 8 °C under sterile conditions after reconstitution. 6 months, -20 to -70 °C under sterile conditions after reconstitution. |
| Handling | Use a manual defrost freezer and avoid repeated freeze-thaw cycles. |
For research use only. Not intended for any clinical use. No products from CD BioSciences may be resold, modified for resale or used to manufacture commercial products without prior written approval from CD BioSciences.