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Home >> Antibodies >> Hamartin Antibody

Hamartin Antibody (F49609)

  Catalog No Formulation Size Price (USD)  
Image F49609-0.4ML In 1X PBS, pH 7.4, with 0.09% sodium azide 0.4 ml 399
Image
F49609-0.08ML In 1X PBS, pH 7.4, with 0.09% sodium azide 0.08 ml 185
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Confocal immunofluorescent analysis of Hamartin antibody with HeLa cells followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used as a nuclear counterstain (blue).
Hamartin antibody immunohistochemistry analysis in formalin fixed and paraffin embedded human skeletal muscle.
Hamartin antibody western blot analysis in MDA-MB231 lysate. Predicted molecular weight: 130~150kDa.
Availability 1-3 business days
Species Reactivity Human
Format Antigen affinity purified
Clonality Polyclonal (rabbit origin)
Isotype Rabbit Ig
Purity Antigen affinity
UniProt Q92574
Applications Western blot : 1:1000
IHC (Paraffin) : 1:10-1:50
Immunofluorescence : 1:10-1:50
Limitations This Hamartin antibody is available for research use only.
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Description

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.

Application Notes

Titration of the Hamartin antibody may be required due to differences in protocols and secondary/substrate sensitivity.

Immunogen

A portion of amino acids 401-430 from the human protein was used as the immunogen for this Hamartin antibody.

Storage

Aliquot the Hamartin antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

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