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Description
Hamartin/TSC1 Recombinant Rabbit mAb (S-2725-166)Product Specification Host Rabbit Antigen Hamartin TSC1 Synonyms Tuberous sclerosis 1 protein; KIAA0243; TSC Immunogen Synthetic Peptide Location Cytoplasm, Lysosome Accession Q92574 Clone Number S 2725 166 Antibody Type Recombinant mAb Isotype IgG Application WB, IHC P Reactivity Hu, Ms Positive Sample HeLa, 293T, IMR 32, mouse skeletal muscle, mouse heart Purification Protein A Concentration 0. 5 mg ml Conjugation Unconjugated Physical Appearance
Product Specification
| Host | Rabbit |
| Antigen | Hamartin/TSC1 |
| Synonyms | Tuberous sclerosis 1 protein; KIAA0243; TSC |
| Immunogen | Synthetic Peptide |
| Location | Cytoplasm, Lysosome |
| Accession | Q92574 |
| Clone Number | S-2725-166 |
| Antibody Type | Recombinant mAb |
| Isotype | IgG |
| Application | WB, IHC-P |
| Reactivity | Hu, Ms |
| Positive Sample | HeLa, 293T, IMR-32, mouse skeletal muscle, mouse heart |
| Purification | Protein A |
| Concentration | 0.5 mg/ml |
| Conjugation | Unconjugated |
| Physical Appearance | Liquid |
| Storage Buffer | PBS, 40% Glycerol, 0.05% BSA, 0.03% Proclin 300 |
| Stability & Storage | 12 months from date of receipt / reconstitution, -20 °C as supplied |
Dilution
| application | dilution | species |
| WB | 1:1000 | Hu, Ms |
| IHC-P | 1:250 | Hu |
Background
Hamartin/TSC1 is a 1,164-amino-acid tumor-suppressor scaffold that, together with tuberin (TSC2), forms the TSC complex that integrates growth-factor, energy and stress signals to dock at the lysosome and switch Rheb-GTP to Rheb-GDP, thereby allosterically locking mTORC1 in its inactive state; TSC1 stabilizes TSC2 via its N-terminal coiled-coil and C-terminal TBC-like domains, and pathogenic TSC1 mutations that disrupt this interaction unleash constitutive mTORC1 signaling, causing tuberous sclerosis complex with multi-organ hamartomas, epilepsy and autism spectrum phenotypes, while systemic or brain-specific TSC1 deletion in mice phenocopies these lesions and makes the protein a key pharmacodynamic node for rapamycin-class drugs.
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