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

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

US Biological

product type :

antibody

product name :

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

catalog :

036421

quantity :

200 ul

clonality :

polyclonal

host :

domestic rabbit

conjugate :

nonconjugated

reactivity :

human

product information

Catalog Number :

036421

Product wo Prefix :

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

Host :

rabbit

Product Type :

Pab

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 Western Blot, Immunohistochemistry, Immunofluorescence, ELISA Recommended Dilution: ELISA1:1,000 Western Blot: 1:100-500 Immunohistochemistry: 1:10-50 Immunofluorescence: 1:10-50 Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Calc Crossreactivity :

Alternate Name :

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

Immunogen :

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

Specificity :

Human

Purity :

Purified by Protein A affinity chromatography.

Form :

Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

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, 0.09% sodium azide. 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.

Calc Applications Abbrev :

E IF IHC WB

Storage Temperature :

-20°C

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).

Picture 1 File Name :

https://usbio-images.r.worldssl.net/prodimages/14/036421_1.jpg