Harmartin is the gene product of tuberous sclerosis-1 (TSC1). It is thought to have a tumor suppression function and also augments the expression of the TSC2 product tuberin by inhibiting its ubiquitination.
Product Information
Format
Ascites
Control
NIH/3T3 cell lysate
Presentation
Ascites fluid. Liquid. Contains no preservative.
Applications
Application
This Anti-Harmartin (TSC1) Antibody is validated for use in WB, IH(P) for the detection of Harmartin (TSC1).
Key Applications
Western Blotting
Immunohistochemistry (Paraffin)
Application Notes
Immunohistochemistry Analysis: A 1:100 dilution of this antibody detected Harmartin in human skin tissue, melanocytes.
FUNCTION:In complex with TSC2, inhibits the nutrient-mediated or growth factor-stimulated phosphorylation of S6K1 and EIF4EBP1 by negatively regulating mTORC1 signaling. Seems not to be required for TSC2 GAP activity towards RHEB. Implicated as a tumor suppressor. Involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling. SUBUNIT STRUCTURE:Interacts with TSC2, leading to stabilize TSC2. In the absence of TSC2, TSC1 self-aggregates. Interacts with DOCK7.SUBCELLULAR LOCATION:Cytoplasm. Membrane; Peripheral membrane protein. Note: At steady state found in association with membranes. TISSUE SPECIFICITY:Highly expressed in skeletal muscle, followed by heart, brain, placenta, pancreas, lung, liver and kidney. Also expressed in embryonic kidney cells. DOMAIN:The C-terminal putative coiled-coil domain is necessary for interaction with TSC2. PTM:Phosphorylation at Ser-505 does not affect interaction with TSC2. Phosphorylated upon DNA damage, probably by ATM or ATR. INVOLVEMENT IN DISEASE:Defects in TSC1 are the cause of tuberous sclerosis complex (TSC) [MIM:191100]. The molecular basis of TSC is a functional impairment 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) [MIM:607341]. 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.
Product Usage Statements
Quality Assurance
Evaluated by Western Blot in NIH/3T3 cell lysate.
Western Blot Analysis: 1:500 dilution of this antibody detected Harmartin in 10 µg of NIH/3T3 cell lysate.
Usage Statement
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Storage and Shipping Information
Storage Conditions
Maintain at -20°C in undiluted aliquots for up to 12 months after date of receipt. Avoid repeated freeze/thaw cycles.
Packaging Information
Material Size
100 µL
原厂资料：
Key Spec Table
Species Reactivity
Key Applications
Host
Format
Antibody Type
H, R
WB, IH(P)
M
Ascites
Monoclonal Antibody
Description
Catalogue Number
05-1113
Replaces
04-426
Description
Anti-Harmartin (TSC1) Antibody
Alternate Names
Tuberous sclerosis 1 protein
tumor suppressor
tuberous sclerosis 1
Background Information
Harmartin is the gene product of tuberous sclerosis-1 (TSC1). It is thought to have a tumor suppression function and also augments the expression of the TSC2 product tuberin by inhibiting its ubiquitination.
Product Information
Format
Ascites
Control
NIH/3T3 cell lysate
Presentation
Ascites fluid. Liquid. Contains no preservative.
Applications
Application
This Anti-Harmartin (TSC1) Antibody is validated for use in WB, IH(P) for the detection of Harmartin (TSC1).
Key Applications
Western Blotting
Immunohistochemistry (Paraffin)
Application Notes
Immunohistochemistry Analysis: A 1:100 dilution of this antibody detected Harmartin in human skin tissue, melanocytes.
FUNCTION:In complex with TSC2, inhibits the nutrient-mediated or growth factor-stimulated phosphorylation of S6K1 and EIF4EBP1 by negatively regulating mTORC1 signaling. Seems not to be required for TSC2 GAP activity towards RHEB. Implicated as a tumor suppressor. Involved in microtubule-mediated protein transport, but this seems to be due to unregulated mTOR signaling. SUBUNIT STRUCTURE:Interacts with TSC2, leading to stabilize TSC2. In the absence of TSC2, TSC1 self-aggregates. Interacts with DOCK7.SUBCELLULAR LOCATION:Cytoplasm. Membrane; Peripheral membrane protein. Note: At steady state found in association with membranes. TISSUE SPECIFICITY:Highly expressed in skeletal muscle, followed by heart, brain, placenta, pancreas, lung, liver and kidney. Also expressed in embryonic kidney cells. DOMAIN:The C-terminal putative coiled-coil domain is necessary for interaction with TSC2. PTM:Phosphorylation at Ser-505 does not affect interaction with TSC2. Phosphorylated upon DNA damage, probably by ATM or ATR. INVOLVEMENT IN DISEASE:Defects in TSC1 are the cause of tuberous sclerosis complex (TSC) [MIM:191100]. The molecular basis of TSC is a functional impairment 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) [MIM:607341]. 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.
Product Usage Statements
Quality Assurance
Evaluated by Western Blot in NIH/3T3 cell lysate.
Western Blot Analysis: 1:500 dilution of this antibody detected Harmartin in 10 µg of NIH/3T3 cell lysate.
Usage Statement
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Storage and Shipping Information
Storage Conditions
Maintain at -20°C in undiluted aliquots for up to 12 months after date of receipt. Avoid repeated freeze/thaw cycles.
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