anti-TSC1, Rb mono

货号： 04-426      产品名称： anti-TSC1, Rb mono   品牌： Millipore 规格： EA 3周到货 生化实验

Anti-TSC1 Antibody, rabbit monoclonal
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REFERENCES
Rhebbing up mTOR: new insights on TSC1 and TSC2, and the pathogenesis of tuberous sclerosis.
Kwiatkowski, David J (2003) Cancer Biol. Ther., 2: 471-6 (2003)
Insulin activation of Rheb, a mediator of mTOR/S6K/4E-BP signaling, is inhibited by TSC1 and 2
Garami, Attila, et al (2003) Mol Cell, 11:1457-66 (2003)
Species Reactivity Key Applications Host Format Antibody Type
Human Only  WB, IH(P) Rabbit Semi-Purified Monoclonal Antibody
Description:
Anti-TSC1 Antibody, rabbit monoclonal
Promotional Text:
Special Shipping Offer on Antibodies
100% Performance Guaranteed
Trade Name:
Upstate (Millipore)
Specificity:
Recognizes the C-terminal domain of human TSC1.
Molecular Weight:
150 kDa
Epitope:
C-terminus
Immunogen:
KLH-conjugated synthetic peptide corresponding to the C-terminal region of human TSC1/Hamartin.
Isotype:
IgG
Species Reactivity:
Human Only
Quality Assurance:
Routinely evaluated by immunoblot.
Storage Conditions:
2 years at -20°C from date of shipment
UniProt Number:
Q92574
Entrez Gene Number:
NM_000368.3
NM_001008567.1
Gene Symbol:
TSC1
TSC
hamartin
LAM
KIAA0243
Hamartin
MGC86987
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Alternate Names:
hamartin; tuberous sclerosis 1 protein
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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Key Applications:
Western Blotting
Immunohistochemistry (Paraffin)
UniProt Summary:
FUNCTION: SwissProt: Q92574 # Implicated as a tumor suppressor. May have a function in vesicular transport. Interaction between TSC1 and TSC2 may facilitate vesicular docking.
SIZE: 1164 amino acids; 129767 Da
SUBUNIT: Interacts with 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:SwissProt: Q92574 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.
DISEASE: SwissProt: Q92574 # Defects in TSC1 are the cause of tuberous sclerosis complex (TSC) [MIM:191100]. 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) [MIM:607341]. FCDBC is a subtype of cortical displasias linked to chronic intractable epilepsy. Cortical dysplasias display a broad -20°C

anti-TSC2, Rb mono

货号： 04-427      产品名称： anti-TSC2, Rb mono   品牌： Millipore 规格： EA 3周到货 生化实验

Anti-TSC2 Antibody, rabbit monoclonal
Species Reactivity Key Applications Host Format Antibody Type
H, M, R  FC, WB, IH(P) Rabbit Semi-Purified Monoclonal Antibody
Description:
Anti-TSC2 Antibody, rabbit monoclonal
Promotional Text:
Special Shipping Offer on Antibodies
100% Performance Guaranteed
Trade Name:
Upstate (Millipore)
Specificity:
Recognizes the C-terminal domain of TSC2.
Molecular Weight:
189 kDa
Epitope:
C-terminal domain
Immunogen:
KLH-conjugated synthetic peptide corresponding to the C-terminal region of human TSC2/Tuberin.
Isotype:
IgG
Species Reactivity:
Human
Mouse
Rat
Quality Assurance:
Routinely evaluated by immunoblot.
Storage Conditions:
2 years at -20°C from date of shipment
UniProt Number:
P49815
Entrez Gene Number:
NM_021055.1
NM_021056.1
NM_000548.3
NM_001077183.1
Gene Symbol:
TSC2
TSC4
FLJ43106
tuberin
LAM
Tuberin
Alternate Names:
tuberous sclerosis 2, tuberin
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.
Key Applications:
Flow Cytometry
Western Blotting
Immunohistochemistry (Paraffin)
Entrez Gene Summary:
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.
UniProt Summary:
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 -20°C