Mouse Anti-Hamartin (TSC1, Tuberous Sclerosis 1) (MaxLight 650)
MaxLight™ 650 is a new Far-IR stable dye conjugate comparable to Alexa Fluor™647, DyLight™649, Cy5™ and offers better labeling efficiency, brighter imaging and increased immunodetection. Absorbance (655nm); Emission (676nm); Extinction Coefficient 250,000.
Tuberous sclerosis (TSC) is an autosomal dominant disease caused by mutations in one of two tumor-suppressor genes, TSC1 and TSC2, resulting in seizures, developmental delays, behavioral problems, benign hamartomas and low grade neoplasms predominantly affecting the central nervous system, kidney, skin, lung, and heart. The dysfunction of either gene products of TSC1 and TSC2, hamartin and tuberin, respectively, may cause the appearance of these tumors. Hamartin and tuberin can interact with each other to form a cytoplasmic complex. When bound, hamartin is able to inhibit the ubiquitination of tuberin, and thus, increasing the cellular level of tuberin and inhibiting cell growth.1 Hamartin also interacts with the ezrin-radixin-moesin family of actin-binding proteins. Hamartin affects cell proliferation by deregulating the G1 phase of the cell cycle.2 Inhibition of hamartin activity results in the loss of cell adhesion. Expression studies has shown that hamartin is colocalized with tuberin in most tissues, including the liver, small and large intestines, prostate, testes, and pancreas.3 However, in the central nervous system, hamartin is mainly localized along neuronal or astrocytic processes, as opposed to tuberin, which is localized to the perinuclear region of the Purkinje cell.4 These studies indicate that hamartin may be important for astrocyte growth control.
Applications
Suitable for use in FLISA, Western Blot and Immunoprecipitation. Other applications not tested.
Recommended Dilutions
Optimal dilutions to be determined by the researcher.
Storage and Stability
Store product at 4°C in the dark. DO NOT FREEZE! Stable at 4°C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight™650 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.
Note: Applications are based on unconjugated antibody.
Immunogen
Recombinant human Hamartin protein
Form
Supplied as a liquid in PBS, pH 7.2. No preservatives added. Labeled with MaxLight™650.
Purity
Purified by Protein A affinity chromatography.
Specificity
Recognizes the ~130-150kD human hamartin protein. No reactivity with the related Tuberin protein. This specificity was confirmed with Western Blot on both Hamartin- and Tuberin-transfected cell lysates. Reactivity was confirmed with a Hamartin-transfected 293 cell line.
