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: 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: 9 A5.3|9 29.12 cM
Cellular Component:  cytoplasm; cytoplasmic vesicle; DNA repair complex; nucleolus; nucleus; spindle
Molecular Function:  1-phosphatidylinositol-3-kinase activity; ATP binding; DNA binding; DNA-dependent protein kinase activity; kinase activity; nucleotide binding; protein binding; protein dimerization activity; protein kinase activity; protein N-terminus binding; protein serine/threonine kinase activity; protein-containing complex binding; transferase activity
Biological Process:  brain development; cell cycle; cell cycle arrest; cellular response to DNA damage stimulus; cellular response to gamma radiation; cellular response to nitrosative stress; cellular response to retinoic acid; cellular response to X-ray; chromosome organization involved in meiotic cell cycle; determination of adult lifespan; DNA damage checkpoint; DNA damage induced protein phosphorylation; DNA repair; double-strand break repair via homologous recombination; establishment of RNA localization to telomere; female gamete generation; female gonad development; female meiotic nuclear division; heart development; histone mRNA catabolic process; histone phosphorylation; immune system process; immunoglobulin production; intrinsic apoptotic signaling pathway in response to DNA damage; lipoprotein catabolic process; male meiotic nuclear division; meiotic telomere clustering; mitotic spindle assembly checkpoint; 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; phosphorylation; 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 histone phosphorylation; positive regulation of neuron apoptotic process; positive regulation of neuron death; positive regulation of response to DNA damage stimulus; 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; regulation of autophagy; regulation of cell cycle; regulation of cellular response to gamma radiation; regulation of microglial cell activation; regulation of telomere maintenance via telomerase; replicative senescence; response to hypoxia; response to ionizing radiation; signal transduction involved in mitotic G2 DNA damage checkpoint; somitogenesis; telomere maintenance; thymus development; V(D)J recombination
Reference #:  Q62388 (UniProtKB)
Alt. Names/Synonyms: A-T mutated homolog; AI256621; ataxia telangiectasia gene mutated in human beings; ataxia telangiectasia mutated; Ataxia telangiectasia mutated homolog; ataxia telangiectasia mutated homolog (human); Atm; C030026E19Rik; Serine-protein kinase ATM
Gene Symbols: Atm
Molecular weight: 349,418 Da
Basal Isoelectric point: 6.43  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  |  Reactome  |  BioGPS  |  KinBase  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene