antibody

Hamartin, ID (TSC1, KIAA0243, TSC, Hamartin, Tuberous sclerosis 1 protein) (APC)

036421-APC

200 ul

polyclonal

domestic rabbit

APC

Hamartin, ID (TSC1, KIAA0243, TSC, Hamartin, Tuberous sclerosis 1 protein) (APC)

rabbit

Pab

APC

Antibodies

200 ul

IgG

Implicated as a tumor suppressor. May have a function in vesicular transport. Interaction between TSC1 and TSC2 may facilitate vesicular docking. Defects in TSC1 are the cause of tuberous sclerosis complex (TSC). The molecular basis of TSC is a functional impairement of the hamartin-tuberin complex. TSC is an autosomal dominant multi-system disorder that affects especially the brain, kidneys, heart, and skin. TSC is characterized by hamartomas (benign overgrowths predominantly of a cell or tissue type that occurs normally in the organ) and hamartias (developmental abnormalities of tissue combination). Clinical symptoms can range from benign hypopigmented macules of the skin to profound mental retardation with intractable seizures to premature death from a variety of disease-associated causes. Defects in TSC1 may be a cause of focal cortical dysplasia of Taylor balloon cell type (FCDBC). FCDBC is a subtype of cortical displasias linked to chronic intractable epilepsy. Cortical dysplasias display a broad spectrum of structural changes, which appear to result from changes in proliferation, migration, differentiation, and apoptosis of neuronal precursors and neurons during cortical development. Applications: Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, FLISA Recommended Dilution: FLISA1:1,000 Western Blot: 1:100-500 Immunohistochemistry: 1:10-50 Immunofluorescence: 1:10-50 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: APC 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.

Hu

TSC1, KIAA0243, TSC, Hamartin, Tuberous sclerosis 1 protein

TSC1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the Center region of human TSC1.

Human

Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).

As reported

Product Type: Pab Isotype: IgG Host: rabbit Source: human Concentration: As reported Form: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC). Purity: Purified by Protein A affinity chromatography. Immunogen: TSC1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the Center region of human TSC1. Specificity: Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.

FL IF IHC WB

4°C Do Not Freeze

Wu, J., et al., J. Cutan. Pathol. 31(5):383-387 (2004). Lewis, J.C., et al., J. Med. Genet. 41(3):203-207 (2004). J, et al., J. Child Neurol. 19(2):102-106 (2004). Murthy, V., et al., J. Biol. Chem. 279(2):1351-1358 (2004). Astrinidis, A., et al., J. Biol. Chem. 278(51):51372-51379 (2003).

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