antibody

ATM Monoclonal Antibody

MBS190053

0.1 mg

420 USD

monoclonal

mouse

nonconjugated

western blot, immunoprecipitation

Antibody

ATM Monoclonal Antibody

ATM

Anti-ATM; ATM

ATM; Serine-protein kinase ATM; serine-protein kinase ATM; AT mutated; A-T mutated; OTTHUMP00000232981; TEL1, telomere maintenance 1, homolog; ataxia telangiectasia mutated; Ataxia telangiectasia mutated

ATM; ATM; AT1; ATA; ATC; ATD; ATE; ATDC; TEL1; TELO1; MGC74674; DKFZp781A0353

ATM_HUMAN

Monoclonal

IgG1 kappa

Mouse

3056

Anti-ATM [Murine MAb recognizes full-length ATM, a 370 kDa nuclear phosphoprotein associated with cell cycle regulation and response to DNA repair.]. This antibody recognizes full-length ATM, a 370 kD nuclear phosphoprotein, which is involved in the autooal recessive disease ataxia telangiectasia (AT). ATM belongs to a novel family of proteins associated with cell cycle regulation and response to DNA repair. The C-terminal region has extensive homology to the catalytic domains of phosphatidylinositol 3-kinases (PI3 kinases). Its usefulness to monitor altered ATM expression in cancer is under active investigations.

100 ug Protein G purified antibody in PBS, pH 7.4

This antibody is stable for at least one (1) year at -70 degree C. Avoid multiple freeze thaw cycles.

Immunoblot, Immunoprecipitation

Immunoblotting: use at 1-10 ug/ml. Immunoprecipitation: use at 1-10 ug/ml. Positive controls: Raji or Akata cells.

Antigen: GST fusion protein expressedin E. coli corresponding to aa 2577-3056 of full-length ATM.

Stabilizer: None. Preservatives: None. Available on request. Dilutions Instructions: Dilute in PBS or medium which is identical to that used in the assay system.

Cancer Research Products

Chen, G and EY-HP Lee (1996) J Biol Chem 271: 33693-33697.

2304971

AAB65827.1

Q13315

350,714 Da

ATM Mediated Phosphorylation Of Repair Proteins Pathway 105865!!ATM Mediated Response To DNA Double-strand Break Pathway 105864!!Apoptosis Pathway 83060!!Apoptosis Pathway 470!!Autodegradation Of The E3 Ubiquitin Ligase COP1 Pathway 160939!!BARD1 Signaling Events Pathway 137959!!Canonical NF-kappaB Pathway 138030!!Cell Cycle Checkpoints Pathway 105739!!Cell Cycle Pathway 198811!!Cell Cycle Pathway 83054

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. [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 plays a role in pre-B cell allelic exclusion, a process leading to expression of a single immunoglobulin heavy chain allele to enforce clonality and monospecific recognition by the B-cell antigen receptor (BCR) expressed on individual B lymphocytes. After the introduction of DNA breaks by the RAG complex on one immunoglobulin allele, acts by mediating a repositioning of the second allele to pericentromeric heterochromatin, preventing accessibility to the RAG complex and recombination of the second allele. 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. Plays a role in replication-dependent histone mRNA degradation. Binds DNA ends. Ref.33 Ref.38 Ref.40 Ref.42. Catalytic activity: ATP + a protein = ADP + a phosphoprotein. Ref.15 Ref.18. Enzyme regulation: Inhibited by wortmannin. Ref.17. Subunit structure: Dimers or tetramers in inactive state. On DNA damage, autophosphorylation dissociates ATM into monomers rendering them catalytically active. Binds 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. Interacts with RAD17; DNA damage promotes the association. Interacts with EEF1E1; the interaction, induced on DNA damage, upregulates TP53. Interacts with DCLRE1C, MYST1, KAT5, OBFC2B, ATMIN and CEP164. Interacts with AP2B1 and AP3B2; the interaction occurs in cytoplasmic vesicles. By similarity. Interacts with TELO2 and TTI1. Interacts with DDX1. Ref.16 Ref.18 Ref.19 Ref.30 Ref.33 Ref.34 Ref.36 Ref.37 Ref.39 Ref.41 Ref.44 Ref.46 Ref.47 Ref.49. Subcellular location: Nucleus. Cytoplasmic vesicle. Note: Primarily nuclear. Found also in endocytic vesicles in association with beta-adaptin. Ref.13 Ref.14. Tissue 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. Ref.17. Domain: The FATC domain is required for interaction with KAT5. Post-translational modification: Phosphorylated by NUAK1/ARK5. Autophosphorylation on Ser-367, Ser-1893, Ser-1981 correlates with DNA damage-mediated activation of the kinase. Ref.12 Ref.20 Ref.21 Ref.23 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29 Ref.31 Ref.32 Ref.33 Ref.35 Ref.39 Ref.40 Ref.43 Ref.45 Ref.48Acetylation, on DNA damage, is required for activation of the kinase activity, dimer-monomer transition, and subsequent autophosphorylation on Ser-1981. Acetylated in vitro by KAT5/TIP60. Involvement in 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.7 Ref.9 Ref.14 Ref.24 Ref.33 Ref.35 Ref.39 Ref.40 Ref.41 Ref.42 Ref.43 Ref.45 Ref.48 Ref.51 Ref.52 Ref.53 Ref.54 Ref.55 Ref.56 Ref.59 Ref.60 Ref.61 Ref.62 Ref.64 Ref.65 Ref.66 Ref.67 Ref.68 Ref.73 Ref.75 Ref.76 Ref.80 Ref.81Note=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.Note=Defects in ATM contribute to B-cell non-Hodgkin lymphomas (BNHL), including mantle cell lymphoma (MCL). Ref.57Note=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.71 Ref.72 Ref.77. Sequence similarities: Belongs to the PI3/PI4-kinase family. ATM subfamily.Contains 1 FAT domain.Contains 1 FATC domain.Contains 1 PI3K/PI4K domain. Sequence caution: The sequence AAA86520.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.The sequence AAA86520.1 differs from that shown. Reason: Probable cloning artifact.The sequence AAI37170.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.The sequence AAI37170.1 differs from that shown. Reason: Probable cloning artifact.The sequence EAW67111.1 differs from that shown. Reason: Erroneous gene model prediction.

0.1 mg

420 USD