Please note that this product will not be available for sale after March 15, 2015. Please select one of the other antibodies against this target. This Anti-TSC2 Antibody, rabbit is validated for use in FC, WB, IH(P) for the detection of TSC2.
Key Applications
Immunohistochemistry (Paraffin)
Flow Cytometry
Western Blotting
Biological Information
Immunogen
KLH-conjugated synthetic peptide corresponding to the C-terminal region of human TSC2/Tuberin.
Mutations in this gene lead to tuberous sclerosis complex. Its gene product is believed to be a tumor suppressor and is able to stimulate specific GTPases. The protein associates with hamartin in a cytosolic complex, possibly acting as a chaperone for hamartin. Alternative splicing results in multiple transcript variants encoding different isoforms.
FUNCTION:SwissProt: P49815 # Implicated as a tumor suppressor. May have a function in vesicular transport, but may also play a role in the regulation of cell growth arrest and in the regulation of transcription mediated by steroid receptors. Interaction between TSC1 and TSC2 may facilitate vesicular docking. Specifically stimulates the intrinsic GTPase activity of the Ras-related protein RAP1A and RAB5. Suggesting a possible mechanism for its role in regulating cellular growth. Mutations in TSC2 leads to constitutive activation of RAP1A in tumors. SIZE:1807 amino acids; 200749 Da SUBUNIT:Interacts with TSC1. May also interact with the adapter molecule RABEP1. The final complex contains TSC2 and RABEP1 linked to RAB5 (Probable). Interacts with HSPA1 and HSPA8. SUBCELLULAR LOCATION:Cytoplasm. Membrane; Peripheral membrane protein. Note=At steady state found in association with membranes. TISSUE SPECIFICITY:Liver, brain, heart, lymphocytes, fibroblasts, biliary epithelium, pancreas, skeletal muscle, kidney, lung and placenta. PTM:Phosphorylation at Ser-1387, Ser-1418 or Ser-1420 does not affect interaction with TSC1. DISEASE:SwissProt: P49815 # Defects in TSC2 are the cause of tuberous sclerosis complex (TSC) [MIM:191100]. The molecular basis of TSC is a functional impairment of the tuberin-hamartin 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 TSC2 are a cause of lymphangioleiomyomatosis (LAM) [MIM:606690]. LAM is a progressive and often fatal lung disease characterized by a diffuse proliferation of abnormal smooth muscle cells in the lungs. It affects almost exclusively young women and can occur as an isolated disorder or in association with tuberous sclerosis complex. SIMILARITY:SwissProt: P49815 ## Contains 1 Rap-GAP domain.
Product Usage Statements
Quality Assurance
Routinely evaluated by immunoblot.
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
2 years at -20°C from date of shipment
Packaging Information
Material Size
100 µL
原厂资料：
Key Spec Table
Species Reactivity
Key Applications
Host
Format
Antibody Type
R, H, M
IH(P), FC, WB
Rb
Semi-Purified
Monoclonal Antibody
Description
Catalogue Number
04-427
Brand Family
Upstate
Trade Name
Upstate
Description
Anti-TSC2 Antibody, rabbit monoclonal
Alternate Names
tuberin
tuberous sclerosis 2
Product Information
Format
Semi-Purified
Applications
Application
Please note that this product will not be available for sale after March 15, 2015. Please select one of the other antibodies against this target. This Anti-TSC2 Antibody, rabbit is validated for use in FC, WB, IH(P) for the detection of TSC2.
Key Applications
Immunohistochemistry (Paraffin)
Flow Cytometry
Western Blotting
Biological Information
Immunogen
KLH-conjugated synthetic peptide corresponding to the C-terminal region of human TSC2/Tuberin.
Mutations in this gene lead to tuberous sclerosis complex. Its gene product is believed to be a tumor suppressor and is able to stimulate specific GTPases. The protein associates with hamartin in a cytosolic complex, possibly acting as a chaperone for hamartin. Alternative splicing results in multiple transcript variants encoding different isoforms.
FUNCTION:SwissProt: P49815 # Implicated as a tumor suppressor. May have a function in vesicular transport, but may also play a role in the regulation of cell growth arrest and in the regulation of transcription mediated by steroid receptors. Interaction between TSC1 and TSC2 may facilitate vesicular docking. Specifically stimulates the intrinsic GTPase activity of the Ras-related protein RAP1A and RAB5. Suggesting a possible mechanism for its role in regulating cellular growth. Mutations in TSC2 leads to constitutive activation of RAP1A in tumors. SIZE:1807 amino acids; 200749 Da SUBUNIT:Interacts with TSC1. May also interact with the adapter molecule RABEP1. The final complex contains TSC2 and RABEP1 linked to RAB5 (Probable). Interacts with HSPA1 and HSPA8. SUBCELLULAR LOCATION:Cytoplasm. Membrane; Peripheral membrane protein. Note=At steady state found in association with membranes. TISSUE SPECIFICITY:Liver, brain, heart, lymphocytes, fibroblasts, biliary epithelium, pancreas, skeletal muscle, kidney, lung and placenta. PTM:Phosphorylation at Ser-1387, Ser-1418 or Ser-1420 does not affect interaction with TSC1. DISEASE:SwissProt: P49815 # Defects in TSC2 are the cause of tuberous sclerosis complex (TSC) [MIM:191100]. The molecular basis of TSC is a functional impairment of the tuberin-hamartin 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 TSC2 are a cause of lymphangioleiomyomatosis (LAM) [MIM:606690]. LAM is a progressive and often fatal lung disease characterized by a diffuse proliferation of abnormal smooth muscle cells in the lungs. It affects almost exclusively young women and can occur as an isolated disorder or in association with tuberous sclerosis complex. SIMILARITY:SwissProt: P49815 ## Contains 1 Rap-GAP domain.
Product Usage Statements
Quality Assurance
Routinely evaluated by immunoblot.
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.
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