antibody

Anti-Hamartin antibody

MBS176028

0.1 mg

245 USD

polyclonal

rabbit

nonconjugated

human, mouse, rat

western blot, immunohistochemistry, immunohistochemistry - paraffin section

Antibody

Anti-Hamartin antibody

Hamartin

Hamartin; tuberous sclerosis 1; Hamartin antibody; kiaa0243 antibody; LAM antibody; TSC antibody; TSC1 antibody; Tsc1 gene antibody; TSC1_HUMAN antibody; Tuberous sclerosis 1 antibody; Tuberous sclerosis 1 protein antibody

Hamartin; Hamartin; hamartin; tumor suppressor; tuberous sclerosis 1 protein; tuberous sclerosis 1; Tuberous sclerosis 1 protein

TSC1

TSC1; TSC1; LAM; TSC; KIAA0243; TSC

TSC1_HUMAN

Polyclonal

IgG

Rabbit

Human, Mouse, Rat

1164

Immunogen affinity purified.

Lyophilized

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Western Blot (WB), Immunohistochemistry (IHC) Paraffin

Western Blot (WB):. Concentration: 0.1-0.5ug/ml. Tested Species: Hu, Rat. Predicted Species: Ms. Antigen Retrieval: n/a . Immunohistrochemistry (IHC) - Paraffin-embedded Section: Concentration: 0.5-1ug/ml. Tested Species: Hu. Predicted Species: Ms,Rat. Antigen Retrieval: By Heat. WB: the detection limit for TSC1 is approximately 5ng/lane under reducing conditions. Tested Species: in-house tested species with positive results. Predicted Species: Species predicted to be fit for the product based on sequence similarities. By Heat: Boiling the paraffin sections in 10mM citrate buffer, pH6.0, for 20mins is required for the staining of fomalin/paraffin sections. Other applications have not been tested. Optimal dilutions should be determined by end users.

Immunogen: A synthetic peptide corresponding to a sequence at the C-terminus of human Hamartin (1150-1164aa QLHIMDYNETHHEHS), identical to the related rat sequence, and different from the related mouse sequence by one amino acid.

Contents: Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Description: Rabbit IgG polyclonal antibody for Hamartin(TSC1) detection. Tested with WB, IHC-P in Human, Mouse, Rat. Background: TSC1(Tuberous Sclerosis 1), also called HAMARTIN or TSC, is a human protein and gene. As part of a comprehensive strategy to identify the gene mutant in tuberous sclerosis-1, van Slegtenhorst et al.(1997) developed an overlapping contig of clones from the 1.4-Mb TSC1 region on chromosome 9. Benvenuto et al.(2000) showed that overexpression of the TSC1 gene in rat fibroblasts inhibits growth and causes changes in cell morphology. Van Slegtenhorst et al.(1998) showed that hamartin and tuberin associate physically in vivo, however, and that the interaction is mediated by predicted coiled-coil domains.

9297077

Q92574.2

Q92574

124,015 Da

AMPK Signaling Pathway (198868); AMPK Signaling Pathway (989139); AMPK Signaling Pathway (992181); Energy Dependent Regulation Of MTOR By LKB1-AMPK Pathway (160972); IGF1R Signaling Cascade Pathway (730346); IRS-mediated Signalling Pathway (106426); IRS-related Events Pathway (106424); IRS-related Events Triggered By IGF1R Pathway (730348); Inhibition Of TSC Complex Formation By PKB Pathway (106431); Insulin Signaling Pathway (198845)

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; 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; cell projection organization and biogenesis; positive regulation of focal adhesion formation; 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; negative regulation of translation; regulation of stress fiber formation; glucose import; 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

0.1 mg

245 USD