Anti-Hamartin / TSC1 Antibody IHC-plus; TSC1; LAM; Tuberous sclerosis 1 protein; Tuberous sclerosis 1; Hamartin; KIAA0243; TSC; Tumor suppressor; Human Hamartin; TSC1

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

Polyclonal

Rabbit

15 amino acid peptide from the middle region of human TSC1 antibody will recognize both isoforms of TSC1

Immunoaffinity Purified

PBS, 0.02% sodium azide.

1 mg/ml (lot specific)

Short term 4 degree C, long term aliquot and store at -20 degree C, avoid freeze thaw cycles. Store undiluted.

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.

Tuberous sclerosis complex (TSC) is an autosomal dominant tumor syndrome caused by mutations in either of the TSC1 or TSC2 tumor suppressor genes. The products of these genes form a protein complex that indirectly decreases the signaling of the mammalian Target of Rapamycin (TOR), an evolutionarily conserved serine/threonine kinase that regulates cell growth and cell cycle through its ability to integrate signals from nutrient levels and growth factors.

Immunohistochemistry (IHC - Paraffin), Immunocytochemistry (ICC), Western Blot (WB)

ICC, IHC-P (2.5 ug/ml), WB (1 ug/ml)
Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary antib...

Anti-TSC1 antibody IHC of human skeletal muscle. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.

Anti-TSC1 antibody IHC of human heart. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.

Immunofluorescence of TSC1 in L1210 cells with TSC1 antibody at 10 ug/ml.

Immunocytochemistry of TSC1 in EL4 cells with TSC1 antibody at 2 ug/ml

Western blot of TSC1 in EL4 cell lysate with TSC1 antibody at 1 ug/ml in the (A) absence and (B) presence of blocking peptide.

124,015 Da

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: Motility/polarity/chemotaxis; Tumor suppressor

Chromosomal Location of Human Ortholog: 9q34

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

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

Biological Process: myelination; protein heterooligomerization; regulation of cell-matrix adhesion; regulation of cell cycle; 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; kidney development; cell cycle arrest; potassium ion transport; negative regulation of TOR signaling pathway; protein stabilization; hippocampus development; rRNA export from nucleus; regulation of stress fiber formation; glucose import; negative regulation of translation; 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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