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Anti-Tuberin/TSC2 Antibody Picoband® Fluoro488 Conjugated

Product Specifications

Background

Tuberous sclerosis 2 protein, also known as TSC2 or Tuberin is a protein that is in humans. The chromosomal location of this gene is 16p13.3. Mutations in this gene lead to tuberous sclerosis complex. Its gene product is believed to be a tumor suppressor and is able to stimulate specific GTPases. The protein associates with hamartin in a cytosolic complex, possibly acting as a chaperone for hamartin. This gene involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling. It stimulates weakly the intrinsic GTPase activity of the Ras-related proteins RAP1A and RAB5 in vitro.

Synonyms

Tuberin; Tuberous sclerosis 2 protein; TSC2; TSC4

Gene Name

TSC2

Gene ID

7249

UniProt

P49815

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins

Immunogen

E.coli-derived human Tuberin recombinant protein (Position: H1611-V1807) . Human Tuberin shares 94% and 90% amino acid (aa) sequences identity with mouse and rat Tuberin, respectively.

Clonality

Polyclonal

Tissue Specificity

Liver, brain, heart, lymphocytes, fibroblasts, biliary epithelium, pancreas, skeletal muscle, kidney, lung and placenta.

Applications

Flow Cytometry

Field of Research

Domain Families, Epigenetics and Nuclear Signaling, Hlh/Leucine Zipper, Transcription, Transcription Factors

Purification

Immunogen affinity purified.

Form

Liquid

Function

In complex with TSC1, inhibits the nutrient-mediated or growth factor-stimulated phosphorylation of S6K1 and EIF4EBP1 by negatively regulating mTORC1 signaling. Acts as a GTPase- activating protein (GAP) for the small GTPase RHEB, a activator of the protein kinase activity of mTORC1. Implicated as a tumor suppressor. Involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling. Stimulates weakly the intrinsic GTPase activity of the Ras-related proteins RAP1A and RAB5 in vitro. Mutations in TSC2 lead to constitutive activation of RAP1A in tumors. .

References & Citations

1. Chu-Shore, C. J., Major, P., Montenegro, M., Thiele, E. Cyst-like tubers are associated with TSC2 and epilepsy in tuberous sclerosis complex. Neurology 72: 1165-1169, 2009. 2. Jansen, F. E., Braams, O., Vincken, K. L., Algra, A., Anbeek, P., Jennekens-Schinkel, A., Halley, D., Zonnenberg, B. A., van den Ouweland, A., van Huffelen, A. C., van Nieuwenhuizen, O., Nellist, M. Overlapping neurologic and cognitive phenotypes in patients with TSC1 or TSC2 mutations. Neurology 70: 908-915, 2008.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Calculated Molecular Weight

200608 MW

Specificity

No cross reactivity with other proteins.

Applications Notes

6

Gene Name Synonym

Tuberin

Subcellular Location

Cytoplasm. Membrane; Peripheral membrane protein. At steady state found in association with membranes.

Sequence Similarities

Contains 1 Rap-GAP domain.

Protein Name

Tuberin

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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