Hamartin, CT (Hamartin, Tuberous sclerosis 1 protein, TSC1, KIAA0243, TSC) (AP) | US Biological 036420-AP product information

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product summary

company name :

US Biological

product type :

antibody

product name :

Hamartin, CT (Hamartin, Tuberous sclerosis 1 protein, TSC1, KIAA0243, TSC) (AP)

catalog :

036420-AP

quantity :

200 ul

clonality :

polyclonal

host :

domestic rabbit

conjugate :

reactivity :

human

product information

Catalog Number :

036420-AP

Product wo Prefix :

Hamartin, CT (Hamartin, Tuberous sclerosis 1 protein, TSC1, KIAA0243, TSC) (AP)

Host :

rabbit

Product Type :

Pab

Conjugate :

Category :

Antibodies

Size1 :

200 ul

Isotype :

IgG

Desc1 :

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 Immunohistochemistry, ELISA Recommended Dilution: ELISA1:1,000 Immunohistochemistry: 1:10-50 Storage and Stability: Store product at 4°C. 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. For maximum recovery of product, centrifuge the original vial prior to removing the cap. Note: Applications are based on unconjugated antibody.

Calc Crossreactivity :

Alternate Name :

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

Immunogen :

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

Specificity :

Human

Purity :

Purified by Protein A affinity chromatography.

Form :

Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phophatase (AP).

Concentration :

As reported

Desc2 :

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 Alkaline Phophatase (AP). Purity: Purified by Protein A affinity chromatography. Immunogen: TSC1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the C-terminal 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.

Calc Applications Abbrev :

E IHC

Storage Temperature :

4°C Do Not Freeze

Reference :

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).