| CatalogCode: | NB100-626 |
| ProductName: | TSC-2 Antibody |
| Product Description: | Rabbit Polyclonal anti-TSC-2 |
| Clonality: | Polyclonal |
| Immunogen: | A synthetic peptide which maps to a region between residues 1750 and the C-terminus (residue 1807) of human Tuberous Sclerosis 2 using the numbering given in Swiss-Prot entry P49815 (GeneID 7249). |
| Specificity: | NB 100-626 is specific for human TSC2 protein. |
| CrossReactivity: | Human. Other species have not been tested. Reactivity with mouse TSC2 is expected owing to high conservation of sequence between this ortholog and human TSC2 at the site to which the epitope maps. |
| Packaging: | 0.1 ml Affinity purified Rabbit antisera. |
| Background: | Tuberous sclerosis is an autosomal dominant human genetic disorder in which distinctive tumors called hamartomas develop. Germline mutations in either TSC1 or TSC2 cause this syndrome, and hamartomas typically display second hit events with loss of the remaining normal allele. Studies initiated in Drosophila have identified a role for the Tsc1 and Tsc2 genes in the regulation of cell and organ size, and genetic interaction studies have placed them in the PI3K-Akt-mTOR-S6K pathway. Biochemical studies have shown that activated Akt phosphorylates TSC2 in the TSC1/TSC2 protein complex, inactivating it; while TSC1/TSC2 has GAP activity for the Rheb GTPase (a member of the ras family), and activated Rheb-GTP activates mTOR. Thus, in cells lacking TSC1 or TSC2 there are increased levels of Rheb-GTP which leads to activation of mTOR, leading to cell size increase and growth. These developments provide enhanced understanding of this signaling pathway and fundamental insights into the pathogenesis of tuberous sclerosis, and open the possibility of treatment for hamartomas by several pharmacologic approaches. (1) |
| Storage: | Store at 4C. Do not freeze. |
| Purity: | Affinity purified |
| Host_Name: | Rabbit |
| Buffer: | Tris-citrate phosphate pH7-8 |
| ListPrice: | 300 |
| AppSummary: | IP, WB |
| SpeciesSummary: | Hu |
| ALTnames: | anti-TSC2 antibody, anti-TSC-2 antibody, anti-Tuberous Sclerosis 2 antibody, anti-Tuberin antibody, anti-LAM antibody |
| ProteinTarget: | TSC-2 Antibody |
| PackageSize: | 0.1 ml |
| GeneralRef: | 1) Cancer Biol Ther. 2003 Sep-Oct;2(5):471-6. |
| NotesMain: | Blocking peptide is available or can be made for this item. Lead time is 3 days to 4 weeks. Price is $ 150/0.25 ml (or 50 ug). Please enquire availability. |
| Gene_Id: | 7249 |
| Omim_ID: | NB110-57632 |
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