Hamartin (TSC1, Tuberous Sclerosis 1)

TSC1, is a tumor suppressor which interacts with tumor suppressor TSC2 (tuberin) to form a cytoplasmic heterodimer. Mutations in either hamartin or tuberin are responsible for tuberous sclerosis (TSC), an autosomal dominant disease characterized by renal dysfunction, seizures, developmental delays, benign hamartomas and low grade neoplasms predominantly affecting the CNS, kidney, lung, skin, and heart. The TSC1/TSC2 complex suppresses cell growth by inhibiting mTOR, with TSC1 acting to inhibit the ubiquitination of TSC2, leading to increased cellular levels of TSC2 and thus enhancing its catalytic activity as a GTPase-activating protein for Rheb. TSC1 and TSC2 are also involved in the G2/M transition of the cell cycle through their interactions with CDK1 and cyclin B1. TSC1 has also been shown to interact with F-actin and ERM (Ezrin-Radixin-Moesin) proteins, implying a role in the modulation of cell adhesion and morphology.\n\nApplications:\nSuitable for use in Flow Cytometry and Western Blot. Other applications not tested.\n\nRecommended Dilution:\nFlow Cytometry (intracellular staining): 2.5ug per 10e6 cells\nWestern Blot: 1ug/ml\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nLyophilized powder may be stored at -20 degrees C. Stable for 12 months at -20 degrees C. Reconstitute with PBS. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Reconstituted product is stable for 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

H1779-02E

Size

100ug

Applications

ELISA, FC, WB

Hosts

Mouse

Reactivities

Hum

Form

Supplied as a powder lyophilized from a solution containing PBS. Reconstitute with sterile PBS to 0.5mg/ml.

P Type

Mab

Purity

Purified by Protein A/G affinity chromatography.

Isotype

IgG2B

Additional Info

Recognizes human TSC1. No cross-reactivity with recombinant human TSC2 (aa550- 850), recombinant human TSC2 (aa1506-1748), or recombinant mouse TSC22 (aa1-143) by direct ELISA.