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

Polyclonal

IgG

Rabbit

This item recognises an epitope within the internal region of the 130kDa tumour suppressor protein, Tumour sclerosis complex 1 (TSC1). Mutation within the TSC1 gene is responsible for Tuberous sclerosis (TSC), an autosomal dominant genetic disorder associated with the development of benign tumors (hamartomas), a condition also induced by mutation of the Tumour sclerosis complex 2 (TSC2) gene.
Many proteins have been shown to play a role in the regulation of the serine/threonine kinase known as target of rapamycin (TOR), a central component in a complex signaling pathway which controls cell proliferation and cell cycle progression. Negative regulation of TOR activity occurs following the over-expression of the TSC1 and TSC2 gene products, hamartin and tuberin. These act by suppressing the phosphorylation of eukaryotic translation initiation factor 4E binding protein 1 (4EBP1) and ribosomal protein S6 kinase (S6K), downstream targets of TOR involved in mRNA translation. Furthermore the TOR activator protein Rheb has been identified as a target of TSC1-TSC2 complexes.

This item has been reported as suitable for use in IHC on mouse EL4 cell cytospins at 2ug/ml.

Purified
Purified IgG - liquid

IgG concentration 1.0mg/ml (lot specific)

Store at 4 degree C or at -20 degree C if preferred. Storage in frost-free freezers is not recommended. This product should be stored undiluted. Avoid repeated freezing and thawing as this may denature the antibody. Should this product contain a precipitate we recommend microcentrifugation before use.
Shelf Life: 18 months from date of despatch.

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.

MBS221310 recognises an epitope within the internal region of the 130kDa tumour suppressor protein, Tumour sclerosis complex 1 (TSC1). Mutation within the TSC1 gene is responsible for Tuberous sclerosis (TSC), an autosomal dominant genetic disorder associated with the development of benign tumors (hamartomas), a condition also induced by mutation of the Tumour sclerosis complex 2 (TSC2) gene. Many proteins have been shown to play a role in the regulation of the serine/threonine kinase known as target of rapamycin (TOR), a central component in a complex signaling pathway which controls cell proliferation and cell cycle progression. Negative regulation of TOR activity occurs following the over-expression of the TSC1 and TSC2 gene products, hamartin and tuberin. These act by suppressing the phosphorylation of eukaryotic translation initiation factor 4E binding protein 1 (4EBP1) and ribosomal protein S6 kinase (S6K), downstream targets of TOR involved in mRNA translation. Furthermore the TOR activator protein Rheb has been identified as a target of TSC1-TSC2 complexes. MBS221310 has been reported as suitable for use in IHC on mouse EL4 cell cytospins at 2ug/ml.

Immunohistology Frozen, Immunohistology Paraffin, Western Blot (WB)

Western Blot: This item detects a band of approximately 130kDa in mouse EL4 cell lysates.
Western Blotting: Maximum Dilution: 1ug/ml

Western blot analysis of whole cell lysate from EL4 mouse lymphoblast cells probed with Rabbit anti Human TSC1 (MBS221310) in the absence (A) or presence (B) of blocking peptide

Immunocytochemical staining of EL4 cells with Rabbit anti Human TSC1 (MBS221310)

124,015 Da

tuberous sclerosis 1

hamartin; tuberous sclerosis 1 protein; tumor suppressor

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; protein complex; growth cone; intracellular membrane-bound organelle; membrane; 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-matrix adhesion; regulation of cell cycle; protein heterooligomerization; 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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