ataxia telangiectasia mutated (includes complementation groups A, C and D)
ataxia telangiectasia mutated protein
human phosphatidylinositol 3-kinase homolog
C and D)
ataxia telangiectasia mutated protein
human phosphatidylinositol 3-kinase homolog
Background Information
ATM (Ataxia Telangiectasia Mutated kinase) and ATR (Ataxia Telangiectasia and Rad3-related kinase) are related kinases that regulate cell cycle checkpoints and DNA repair. ATM is activated in response to DNA damage and serves to arrest further cell division before the damage can be repaired. Mutation in the ATM gene results in the autosomal recessive disease ataxia telangiectasia (AT). The identified substrates for ATM are p53, p95/NBS1, MDM2, Chk2, BRCA1, CtIP, 4E-BP1 and Chk1. ATM activates p53, increasing p21/Cip1/Waf1 levels, thus blocking activation of Cdk2. That results in Rb hypophosphorylation and blockage of the G1/S transition. Separately, ATM also phosphorylates and activates Chk1, which phosphorylates Cdc25C. This inactivates Cdc25C and prevents it from dephosphorylating the inhibitory phosphotyrosine residue on cdc2/Cdk1, thus preventing the G2/M transition. The complex phenotype of cells derived from patients with AT suggests that ATM has additional cellular substrates. In unirradiated cells, ATM is present as an inactive homodimer or multimer. Double-stranded breaks in DNA caused by ionizing radiation cause rapid ATM kinase activation through dissociation of this complex and ATM autophosphorylation at Ser1981.
Product Information
Format
Culture Supernatant
Control
HEK293 cell lysate.
Presentation
Unpurified rabbit monoclonal IgG in buffer containing 50 mM Tris-Glycine (pH 7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.
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-ATM Antibody, clone Y170, Rabbit is validated for use in IC, IF, IH, IH(P), IP, WB for the detection of ATM.
Key Applications
Western Blotting
Immunocytochemistry
Immunofluorescence
Immunohistochemistry
Immunohistochemistry (Paraffin)
Immunoprecipitation
Application Notes
Immunocytochemistry:
A 1:100-250 dilution of a previous lot of this antibody was used on immunocytochemistry.
Immunohistochemistry:
A 1:250 dilution of a previous lot of this antibody was used on immunohistochemistry.
Immunoprecipitation:
A 1:50 dilution of a previous lot of this antibody was used on immunoprecipitation.
Biological Information
Immunogen
Synthetic peptide corresponding to the FAT domain encompassing the area of Ser1981 of human ATM.
Epitope
area encompassing the autophosphorylation site at Ser1981 in the FAT Domain
Clone
Y170
Host
Rabbit
Specificity
This antibody recognizes ATM irrespective of phosphorylation on Ser1981.
Isotype
IgG
Species Reactivity
Human
Species Reactivity Note
Proven to react with human. Not expected to react with mouse or rat.
The protein encoded by this gene belongs to the PI3/PI4-kinase family. This protein is an important cell cycle
checkpoint kinase that phosphorylates; thus, it functions as a regulator of a wide variety of downstream proteins, including tumor suppressor proteins p53 and BRCA1, checkpoint kinase CHK2, checkpoint proteins RAD17 and RAD9, and DNA repair protein NBS1. This protein and the closely related kinase ATR are thought to be master controllers of cell cycle checkpoint signaling pathways that are required for cell response to DNA damage and for genome stability. Mutations in this gene are associated with ataxia telangiectasia, an autosomal recessive disorder. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq].
FUNCTION:Serine/threonine protein kinase which activates checkpoint signaling upon double strand breaks (DSBs), apoptosis and genotoxic stresses such as ionizing ultraviolet A light (UVA), thereby acting as a DNA damage sensor. Recognizes the substrate consensus sequence [ST]-Q. Phosphorylates 'Ser-139' of histone variant H2AX/H2AFX at double strand breaks (DSBs), thereby regulating DNA damage response mechanism. Also involved in signal transduction and cell cycle control. May function as a tumor suppressor. Necessary for activation of ABL1 and SAPK. Phosphorylates p53/TP53, FANCD2, NFKBIA, BRCA1, CTIP, nibrin (NBN), TERF1, RAD9 and DCLRE1C. May play a role in vesicle and/or protein transport. Could play a role in T-cell development, gonad and neurological function.
Catalytic activity ATP + a protein = ADP + a phosphoprotein.
Enzyme regulation Inhibited by wortmannin. Ref.14
STRUCTURE:Exists in monomeric and tetrameric state. Binds DNA ends, p53/TP53, ABL1, BRCA1, NBN/nibrin and TERF1. Part of the BRCA1-associated genome surveillance complex (BASC), which contains BRCA1, MSH2, MSH6, MLH1, ATM, BLM, PMS2 and the RAD50-MRE11-NBN protein complex. This association could be a dynamic process changing throughout the cell cycle and within subnuclear domains. DNA damage promotes association with RAD17. Interacts with EEF1E1. This interaction, which takes place independently of TP53, is induced by DNA damage that may occur during genotoxic stress or cell growth. Interacts with DCLRE1C. Interacts with MYST1. Interacts with HTATIP. Interacts with OBFC2B. Interacts with ATMIN. Ref.13 Ref.15 Ref.16 Ref.27 Ref.30 Ref.32 Ref.33 Ref.34 Ref.35 Ref.38
SUBCELLULAR LOCATION:Nucleus. Cytoplasmic vesicle. Note= Primarily nuclear. Found also in endocytic vesicles in association with beta-adaptin. Ref.10 Ref.11
SPECIFICITY:Found in pancreas, kidney, skeletal muscle, liver, lung, placenta, brain, heart, spleen, thymus, testis, ovary, small intestine, colon and leukocytes.
INDUCTION:By ionizing radiation.
DOMAIN:The FATC domain is required for interaction with HTATIP.
PTM:Phosphorylated by ARK5. Autophosphorylated on Ser-1981 upon DNA damage. Ref.34 Ref.9 Ref.17 Ref.18 Ref.20 Ref.22 Ref.23 Ref.24 Ref.25 Ref.26 Ref.28 Ref.29 Ref.31 Ref.36 Ref.37
Acetylated by HTATIP upon DNA damage; which is required for autophosphorylation and subsequent activation.
DISEASE:Defects in ATM are the cause of ataxia telangiectasia (AT) [MIM:208900]; also known as Louis-Bar syndrome, which includes four complementation groups: A, C, D and E. This rare recessive disorder is characterized by progressive cerebellar ataxia, dilation of the blood vessels in the conjunctiva and eyeballs, immunodeficiency, growth retardation and sexual immaturity. AT patients have a strong predisposition to cancer; about 30% of patients develop tumors, particularly lymphomas and leukemias. Cells from affected individuals are highly sensitive to damage by ionizing radiation and resistant to inhibition of DNA synthesis following irradiation. Ref.34 Ref.35 Ref.11 Ref.31 Ref.36 Ref.37 Ref.4 Ref.7 Ref.21 Ref.39 Ref.40 Ref.41 Ref.42 Ref.43 Ref.44 Ref.47 Ref.48 Ref.49 Ref.50 Ref.52 Ref.53 Ref.54 Ref.55 Ref.56 Ref.61 Ref.63 Ref.64 Ref.68 Ref.69
Defects in ATM contribute to T-cell acute lymphoblastic leukemia (TALL) and T-prolymphocytic leukemia (TPLL). TPLL is characterized by a high white blood cell count, with a predominance of prolymphocytes, marked splenomegaly, lymphadenopathy, skin lesions and serous effusion. The clinical course is highly aggressive, with poor response to chemotherapy and short survival time. TPLL occurs both in adults as a sporadic disease and in younger AT patients.
Defects in ATM contribute to B-cell non-Hodgkin lymphomas (BNHL), including mantle cell lymphoma (MCL). Ref.45
Defects in ATM contribute to B-cell chronic lymphocytic leukemia (BCLL). BCLL is the commonest form of leukemia in the elderly. It is characterized by the accumulation of mature CD5+ B lymphocytes, lymphadenopathy, immunodeficiency and bone marrow failure. Ref.59 Ref.60 Ref.65
SIMILARITY:Belongs to the PI3/PI4-kinase family. ATM subfamily.
Contains 1 FAT domain.
Contains 1 FATC domain.
Contains 1 PI3K/PI4K domain.
Product Usage Statements
Quality Assurance
Routinely evaluated by Western Blot on HEK293 cell lysate.
Western Blot Analysis:
A 1:2,500-1:5,000 dilution of this lot was used to detect ATM in HEK293 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
Stable for 1 year at -20ºC from date of receipt.
Handling Recommendations: Upon receipt, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance. Note: Variability in freezer temperatures below -20°C may cause glycerol containing solutions to become frozen during storage.
Packaging Information
Material Size
100 µL
原厂资料：
Key Spec Table
Species Reactivity
Key Applications
Host
Format
Antibody Type
H
WB, ICC, IF, IHC, IH(P), IP
Rb
Culture Supernatant
Monoclonal Antibody
Description
Catalogue Number
04-200
Brand Family
Upstate
Trade Name
Upstate
Description
Anti-ATM Antibody, clone Y170, rabbit monoclonal
Alternate Names
serine-protein kinase ATM
A-T, mutated
AT mutated
TEL1
telomere maintenance 1, homolog
ataxia telangiectasia mutated
ataxia telangiectasia mutated (includes complementation groups A, C and D)
ataxia telangiectasia mutated protein
human phosphatidylinositol 3-kinase homolog
C and D)
ataxia telangiectasia mutated protein
human phosphatidylinositol 3-kinase homolog
Background Information
ATM (Ataxia Telangiectasia Mutated kinase) and ATR (Ataxia Telangiectasia and Rad3-related kinase) are related kinases that regulate cell cycle checkpoints and DNA repair. ATM is activated in response to DNA damage and serves to arrest further cell division before the damage can be repaired. Mutation in the ATM gene results in the autosomal recessive disease ataxia telangiectasia (AT). The identified substrates for ATM are p53, p95/NBS1, MDM2, Chk2, BRCA1, CtIP, 4E-BP1 and Chk1. ATM activates p53, increasing p21/Cip1/Waf1 levels, thus blocking activation of Cdk2. That results in Rb hypophosphorylation and blockage of the G1/S transition. Separately, ATM also phosphorylates and activates Chk1, which phosphorylates Cdc25C. This inactivates Cdc25C and prevents it from dephosphorylating the inhibitory phosphotyrosine residue on cdc2/Cdk1, thus preventing the G2/M transition. The complex phenotype of cells derived from patients with AT suggests that ATM has additional cellular substrates. In unirradiated cells, ATM is present as an inactive homodimer or multimer. Double-stranded breaks in DNA caused by ionizing radiation cause rapid ATM kinase activation through dissociation of this complex and ATM autophosphorylation at Ser1981.
Product Information
Format
Culture Supernatant
Control
HEK293 cell lysate.
Presentation
Unpurified rabbit monoclonal IgG in buffer containing 50 mM Tris-Glycine (pH 7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.
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-ATM Antibody, clone Y170, Rabbit is validated for use in IC, IF, IH, IH(P), IP, WB for the detection of ATM.
Key Applications
Western Blotting
Immunocytochemistry
Immunofluorescence
Immunohistochemistry
Immunohistochemistry (Paraffin)
Immunoprecipitation
Application Notes
Immunocytochemistry:
A 1:100-250 dilution of a previous lot of this antibody was used on immunocytochemistry.
Immunohistochemistry:
A 1:250 dilution of a previous lot of this antibody was used on immunohistochemistry.
Immunoprecipitation:
A 1:50 dilution of a previous lot of this antibody was used on immunoprecipitation.
Biological Information
Immunogen
Synthetic peptide corresponding to the FAT domain encompassing the area of Ser1981 of human ATM.
Epitope
area encompassing the autophosphorylation site at Ser1981 in the FAT Domain
Clone
Y170
Host
Rabbit
Specificity
This antibody recognizes ATM irrespective of phosphorylation on Ser1981.
Isotype
IgG
Species Reactivity
Human
Species Reactivity Note
Proven to react with human. Not expected to react with mouse or rat.
The protein encoded by this gene belongs to the PI3/PI4-kinase family. This protein is an important cell cycle
checkpoint kinase that phosphorylates; thus, it functions as a regulator of a wide variety of downstream proteins, including tumor suppressor proteins p53 and BRCA1, checkpoint kinase CHK2, checkpoint proteins RAD17 and RAD9, and DNA repair protein NBS1. This protein and the closely related kinase ATR are thought to be master controllers of cell cycle checkpoint signaling pathways that are required for cell response to DNA damage and for genome stability. Mutations in this gene are associated with ataxia telangiectasia, an autosomal recessive disorder. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq].
FUNCTION:Serine/threonine protein kinase which activates checkpoint signaling upon double strand breaks (DSBs), apoptosis and genotoxic stresses such as ionizing ultraviolet A light (UVA), thereby acting as a DNA damage sensor. Recognizes the substrate consensus sequence [ST]-Q. Phosphorylates 'Ser-139' of histone variant H2AX/H2AFX at double strand breaks (DSBs), thereby regulating DNA damage response mechanism. Also involved in signal transduction and cell cycle control. May function as a tumor suppressor. Necessary for activation of ABL1 and SAPK. Phosphorylates p53/TP53, FANCD2, NFKBIA, BRCA1, CTIP, nibrin (NBN), TERF1, RAD9 and DCLRE1C. May play a role in vesicle and/or protein transport. Could play a role in T-cell development, gonad and neurological function.
Catalytic activity ATP + a protein = ADP + a phosphoprotein.
Enzyme regulation Inhibited by wortmannin. Ref.14
STRUCTURE:Exists in monomeric and tetrameric state. Binds DNA ends, p53/TP53, ABL1, BRCA1, NBN/nibrin and TERF1. Part of the BRCA1-associated genome surveillance complex (BASC), which contains BRCA1, MSH2, MSH6, MLH1, ATM, BLM, PMS2 and the RAD50-MRE11-NBN protein complex. This association could be a dynamic process changing throughout the cell cycle and within subnuclear domains. DNA damage promotes association with RAD17. Interacts with EEF1E1. This interaction, which takes place independently of TP53, is induced by DNA damage that may occur during genotoxic stress or cell growth. Interacts with DCLRE1C. Interacts with MYST1. Interacts with HTATIP. Interacts with OBFC2B. Interacts with ATMIN. Ref.13 Ref.15 Ref.16 Ref.27 Ref.30 Ref.32 Ref.33 Ref.34 Ref.35 Ref.38
SUBCELLULAR LOCATION:Nucleus. Cytoplasmic vesicle. Note= Primarily nuclear. Found also in endocytic vesicles in association with beta-adaptin. Ref.10 Ref.11
SPECIFICITY:Found in pancreas, kidney, skeletal muscle, liver, lung, placenta, brain, heart, spleen, thymus, testis, ovary, small intestine, colon and leukocytes.
INDUCTION:By ionizing radiation.
DOMAIN:The FATC domain is required for interaction with HTATIP.
PTM:Phosphorylated by ARK5. Autophosphorylated on Ser-1981 upon DNA damage. Ref.34 Ref.9 Ref.17 Ref.18 Ref.20 Ref.22 Ref.23 Ref.24 Ref.25 Ref.26 Ref.28 Ref.29 Ref.31 Ref.36 Ref.37
Acetylated by HTATIP upon DNA damage; which is required for autophosphorylation and subsequent activation.
DISEASE:Defects in ATM are the cause of ataxia telangiectasia (AT) [MIM:208900]; also known as Louis-Bar syndrome, which includes four complementation groups: A, C, D and E. This rare recessive disorder is characterized by progressive cerebellar ataxia, dilation of the blood vessels in the conjunctiva and eyeballs, immunodeficiency, growth retardation and sexual immaturity. AT patients have a strong predisposition to cancer; about 30% of patients develop tumors, particularly lymphomas and leukemias. Cells from affected individuals are highly sensitive to damage by ionizing radiation and resistant to inhibition of DNA synthesis following irradiation. Ref.34 Ref.35 Ref.11 Ref.31 Ref.36 Ref.37 Ref.4 Ref.7 Ref.21 Ref.39 Ref.40 Ref.41 Ref.42 Ref.43 Ref.44 Ref.47 Ref.48 Ref.49 Ref.50 Ref.52 Ref.53 Ref.54 Ref.55 Ref.56 Ref.61 Ref.63 Ref.64 Ref.68 Ref.69
Defects in ATM contribute to T-cell acute lymphoblastic leukemia (TALL) and T-prolymphocytic leukemia (TPLL). TPLL is characterized by a high white blood cell count, with a predominance of prolymphocytes, marked splenomegaly, lymphadenopathy, skin lesions and serous effusion. The clinical course is highly aggressive, with poor response to chemotherapy and short survival time. TPLL occurs both in adults as a sporadic disease and in younger AT patients.
Defects in ATM contribute to B-cell non-Hodgkin lymphomas (BNHL), including mantle cell lymphoma (MCL). Ref.45
Defects in ATM contribute to B-cell chronic lymphocytic leukemia (BCLL). BCLL is the commonest form of leukemia in the elderly. It is characterized by the accumulation of mature CD5+ B lymphocytes, lymphadenopathy, immunodeficiency and bone marrow failure. Ref.59 Ref.60 Ref.65
SIMILARITY:Belongs to the PI3/PI4-kinase family. ATM subfamily.
Contains 1 FAT domain.
Contains 1 FATC domain.
Contains 1 PI3K/PI4K domain.
Product Usage Statements
Quality Assurance
Routinely evaluated by Western Blot on HEK293 cell lysate.
Western Blot Analysis:
A 1:2,500-1:5,000 dilution of this lot was used to detect ATM in HEK293 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
Stable for 1 year at -20ºC from date of receipt.
Handling Recommendations: Upon receipt, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance. Note: Variability in freezer temperatures below -20°C may cause glycerol containing solutions to become frozen during storage.
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