ATM 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 DYRK2, CHEK2, p53/TP53, FANCD2, NFKBIA, BRCA1, CTIP, nibrin (NBN), TERF1, RAD9, UBQLN4 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. Phosphorylation of DYRK2 in nucleus in response to genotoxic stress prevents its MDM2-mediated ubiquitination and subsequent proteasome degradation. Phosphorylates ATF2 which stimulates its function in DNA damage response. Belongs to the PI3/PI4-kinase family. ATM subfamily. Found in pancreas, kidney, skeletal muscle, liver, lung, placenta, brain, heart, spleen, thymus, testis, ovary, small intestine, colon and leukocytes. Note: This description may include information from UniProtKB.
Protein type: ATM subfamily; ATYPICAL group; DNA repair, damage; EC; Kinase, protein; PIKK family; Protein kinase, Ser/Thr (non-receptor); Protein kinase, atypical; Tumor suppressor
Chromosomal Location of human Ortholog: 11q22.3
Cellular Component:  centrosome; chromosome, telomeric region; cytoplasm; cytoplasmic vesicle; cytosol; DNA repair complex; intracellular membrane-bounded organelle; nucleolus; nucleoplasm; nucleus; peroxisomal matrix; spindle
Molecular Function:  1-phosphatidylinositol-3-kinase activity; ATP binding; DNA binding; DNA-dependent protein kinase activity; identical protein binding; protein binding; protein serine kinase activity; protein serine/threonine kinase activity; protein-containing complex binding
Biological Process:  brain development; cellular response to gamma radiation; cellular response to nitrosative stress; cellular response to reactive oxygen species; cellular response to retinoic acid; cellular response to X-ray; cellular senescence; determination of adult lifespan; DNA damage checkpoint signaling; DNA damage response; DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest; double-strand break repair; double-strand break repair via homologous recombination; double-strand break repair via nonhomologous end joining; establishment of protein-containing complex localization to telomere; establishment of RNA localization to telomere; female meiotic nuclear division; heart development; histone mRNA catabolic process; intrinsic apoptotic signaling pathway in response to DNA damage; lipoprotein catabolic process; male meiotic nuclear division; meiotic telomere clustering; mitotic G2 DNA damage checkpoint signaling; mitotic spindle assembly checkpoint signaling; multicellular organism growth; negative regulation of B cell proliferation; negative regulation of telomere capping; negative regulation of TORC1 signaling; neuron apoptotic process; oocyte development; ovarian follicle development; peptidyl-serine autophosphorylation; peptidyl-serine phosphorylation; pexophagy; phosphatidylinositol-3-phosphate biosynthetic process; positive regulation of apoptotic process; positive regulation of cell adhesion; positive regulation of cell migration; positive regulation of DNA catabolic process; positive regulation of DNA damage response, signal transduction by p53 class mediator; positive regulation of gene expression; positive regulation of neuron apoptotic process; positive regulation of telomerase catalytic core complex assembly; positive regulation of telomere maintenance via telomerase; positive regulation of telomere maintenance via telomere lengthening; positive regulation of transcription by RNA polymerase II; post-embryonic development; pre-B cell allelic exclusion; protein autophosphorylation; protein phosphorylation; reciprocal meiotic recombination; regulation of apoptotic process; regulation of autophagy; regulation of cell cycle; regulation of cellular response to heat; regulation of microglial cell activation; regulation of signal transduction by p53 class mediator; regulation of telomerase activity; regulation of telomere maintenance via telomerase; replicative senescence; response to hypoxia; response to ionizing radiation; signal transduction; signal transduction in response to DNA damage; somitogenesis; telomere maintenance; thymus development; V(D)J recombination
Disease: Ataxia-telangiectasia; Breast Cancer
Reference #:  Q13315 (UniProtKB)
Alt. Names/Synonyms: A-T mutated; AT mutated; AT1; ATA; Ataxia telangiectasia mutated; ataxia telangiectasia mutated (includes complementation groups A, C and D); ATC; ATD; ATDC; ATE; ATM; ATM serine/threonine kinase; DKFZp781A0353; human phosphatidylinositol 3-kinase homolog; MGC74674; Serine-protein kinase ATM; TEL1; TEL1, telomere maintenance 1, homolog; TELO1
Gene Symbols: ATM
Molecular weight: 350,687 Da
Basal Isoelectric point: 6.39  Predict pI for various phosphorylation states
CST Pathways:  Apoptosis Regulation  |  G1/S Checkpoint  |  G2/M DNA Damage Checkpoint  |  Mitochondrial Control of Apoptosis  |  NF-kB Signaling  |  Protein Acetylation
Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology® Total Proteins
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Cross-references to other databases:  STRING  |  cBioPortal  |  CCLE  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  KinBase  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  Ensembl Protein