Purified 抗 Hamartin (TSC1) 抗体

Purified anti-Hamartin (TSC1) Antibody详细如下：

Description ：Tuberous sclerosis complex (TSC) is an autosomal dominant disorder that is due toinactivating mutations in TSC1 (also known as hamartin) or TSC2 (also known as tuberin). TSCis featured by benign tumors (hamartomas) involving multiple organ systems. TSC1, TSC2 andTBC1 domain family member 7 (TBC1D7) form the TSC protein complex that acts as aninhibitor of the mechanistic target of rapamycin (mTOR) signaling, which in turn plays a criticalrole in regulating cell growth, proliferation, autophagy and protein and lipid synthesis. The TSCprotein complex controls the activity of mTORC1 via RAS homologue enriched in brain (RHEB).mTORC1 downstream targets include phospho‑p70 ribosomal S6 kinase, phospho-ribosomalprotein S6 and phospho‑4EBP1 (eukaryotic translation initiation factor 4E‑binding protein 1).Activation of mTORC1 enhances protein translation, and hyperactivation of mTORC1 has beendiscovered to lead to extensive metabolic reprogramming, including effects on glycolysis,autophagy, nucleotide biosynthesis and lipid biosynthesis. In many cases, this reprogrammingleads to vulnerabilities that induce cell death indicating that the altered metabolism of cells withmTORC1 hyperactivation will provide therapeutic opportunities. By contrast, inhibition ofmTORC1 with allosteric inhibitors, such as sirolimus and everolimus, restores TSC2‑deficientcells to metabolic homeostasis and may thereby ‘protect’ them from cell death.

In addition to the canonical TSC–RHEB–mTORC1 pathway, there is evidence of non-canonicalpathways. These include targets of TSC1 that are TSC2 independent, targets of TSC1 andTSC2 that are RHEB independent and targets of RHEB that are mTORC1 independent.

Verified Reactivity ：Human, Mouse

Antibody Type ：Monoclonal

Host Species ：Mouse

Immunogen ：Partial human TSC1 recombinant protein (480-666 a.a.)

Formulation ：Phosphate-buffered solution, pH 7.2, containing 0.09% sodium azide.

Preparation ：The antibody was purified by affinity chromatography.

Concentration ：0.5 mg/ml

Storage & Handling ：The antibody solution should be stored undiluted between 2°C and 8°C.

Application ：WB - Quality tested

Recommended Usage ：Each lot of this antibody is quality control tested by Western blotting. For Western blotting, thesuggested use of this reagent is 0.5 - 5.0 µg per ml. It is recommended that the reagent be titratedfor optimal performance for each application.