Hamartin; TSC1: KIAA0243; LAM; MGC86987; TSC

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Affinity Purification

1mg/ml (lot specific)

Store at -20 degree C (regular) or -80 degree C (long term). Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Small volumes of anti-TSC1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

TSC1, also named as KIAA0243 and TSC, is implicated as a tumor suppressor. It is involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling. In complex with TSC2, TSC1 inhibits the nutrient-mediated or growth factor-stimulated phosphorylation of S6K1 and EIF4EBP1 by negatively regulating mTORC1 signaling. It seems not to be required for TSC2 GAP activity towards RHEB. It is involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling. Defects in TSC1 are the cause of tuberous sclerosis complex (TSC). Defects in TSC1 may be a cause of focal cortical dysplasia of Taylor balloon cell type (FCDBC). The antibody is specific to TSC1.

Polyclonal

Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

WB: 1:500 - 1:1000
IHC: 1:50 - 1:100
IF: 1:20 - 1:50

Western blot analysis of extracts of mouse brain, using TSC1 antibody.
Secondary antibody: HRP Goat Anti-Mouse IgG (H+L) (MBS128212) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.

1164

tuberous sclerosis 1

hamartin; tumor suppressor; tuberous sclerosis 1 protein

Hamartin

KIAA0243; TSC??[Similar Products]

TSC1_HUMAN

This gene encodes a growth inhibitory protein thought to play a role in the stabilization of tuberin. Mutations in this gene have been associated with tuberous sclerosis. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2009]

TSC1: tuberous sclerosis protein 1 (TSC1). May have tumor suppressor activity. Together with tuberin (TSC2), inhibits mammalian target of rapamycin (mTOR)-mediated signaling to eukaryotic initiation factor 4E-binding protein 1 (4E-BP1) and ribosomal protein S6 kinase. Regulated by the phosphatidylinositol 3-kinase/Akt pathway and phosphorylation of tuberin. Phosphorylated by cyclin-dependent kinase 1/cyclin B during the cell cycle.

Protein type: Tumor suppressor; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 9q34

Cellular Component: TSC1-TSC2 complex; growth cone; protein complex; membrane; intracellular membrane-bound organelle; lamellipodium; cytoplasm; plasma membrane; actin filament; cell cortex; cytosol

Molecular Function: protein binding; chaperone binding; protein N-terminus binding; GTPase activating protein binding; GTPase regulator activity

Biological Process: myelination; regulation of cell cycle; protein heterooligomerization; regulation of cell-matrix adhesion; cell-matrix adhesion; negative regulation of insulin receptor signaling pathway; negative regulation of cell size; regulation of phosphoprotein phosphatase activity; cardiac muscle cell differentiation; response to insulin stimulus; regulation of translation; negative regulation of cell proliferation; positive regulation of focal adhesion formation; cell projection organization and biogenesis; synapse organization and biogenesis; cell cycle arrest; kidney development; potassium ion transport; negative regulation of TOR signaling pathway; protein stabilization; hippocampus development; rRNA export from nucleus; negative regulation of translation; glucose import; regulation of stress fiber formation; neural tube closure; insulin receptor signaling pathway; cerebral cortex development; regulation of protein kinase activity

Disease: Lymphangioleiomyomatosis; Focal Cortical Dysplasia Of Taylor; Tuberous Sclerosis 1

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