ATM - Serine-protein kinase ATM - human protein (Function)
 
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ATM »  Serine-protein kinase ATM   [ EC 2.7.11.1 ]
 
Protein also known as:  Ataxia telangiectasia mutated (A-T mutated).
Gene name:  ATM
Family name: PI3/PI4-kinase » ATM
Entry whose protein(s) existence is based on evidence at protein level
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1 318 1
GENE REF ISO

Function

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Overview 
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 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.  
9
  • UniProtKB
GO molecular function 
1-phosphatidylinositol-3-kinase activitydefinition[GO:0016303]  
1
  • UniProtKB
ATP bindingdefinition[GO:0005524]  
1
  • UniProtKB KW
DNA bindingdefinition[GO:0003677]  
1
  • UniProtKB KW
DNA-dependent protein kinase activitydefinition[GO:0004677]  
1
  • BHF-UCL
Histone serine kinase activitydefinition[GO:0035174] silver  
1
  • Ortholog Compara
Protein bindingdefinition[GO:0005515]  
13
  • IntAct
  • UniProtKB
Protein complex bindingdefinition[GO:0032403]  
1
  • BHF-UCL
Protein dimerization activitydefinition[GO:0046983]  
1
  • BHF-UCL
Protein N-terminus bindingdefinition[GO:0047485]  
1
  • UniProtKB
Protein serine/threonine kinase activitydefinition[GO:0004674]  
3
  • UniProtKB
  • BHF-UCL
GO biological process 
Brain developmentdefinition[GO:0007420] silver  
1
  • Ortholog Compara
Cell cycle arrestdefinition[GO:0007050]  
1
  • BHF-UCL
Cellular response to DNA damage stimulusdefinition[GO:0006974]  
2
  • UniProtKB
  • BHF-UCL
Cellular response to gamma radiationdefinition[GO:0071480]  
1
  • BHF-UCL
DNA damage induced protein phosphorylationdefinition[GO:0006975]  
1
  • BHF-UCL
Heart developmentdefinition[GO:0007507] silver  
1
  • Ortholog Compara
Histone mRNA catabolic processdefinition[GO:0071044]  
1
  • UniProtKB
Histone-serine phosphorylationdefinition[GO:0035404] silver  
1
  • Ortholog Compara
Intrinsic apoptotic signaling pathway in response to DNA damagedefinition[GO:0008630] silver  
1
  • Ortholog Compara
Lipoprotein catabolic processdefinition[GO:0042159] silver  
1
  • Ortholog Compara
Mitotic spindle assembly checkpointdefinition[GO:0007094]  
1
  • UniProtKB
Negative regulation of B cell proliferationdefinition[GO:0030889]  
1
  • UniProtKB
Neuron apoptotic processdefinition[GO:0051402] silver  
1
  • Ortholog Compara
Oocyte developmentdefinition[GO:0048599] silver  
1
  • Ortholog Compara
Peptidyl-serine phosphorylationdefinition[GO:0018105]  
1
  • BHF-UCL
Phosphatidylinositol-3-phosphate biosynthetic processdefinition[GO:0036092]  
1
  • GOC
Positive regulation of apoptotic processdefinition[GO:0043065]  
1
  • UniProtKB
Positive regulation of DNA damage response, signal transduction by p53 class mediatordefinition[GO:0043517]  
1
  • BHF-UCL
Positive regulation of neuron apoptotic processdefinition[GO:0043525] silver  
1
  • Ortholog Compara
Pre-B cell allelic exclusiondefinition[GO:0002331]  
1
  • Ortholog Curator
Protein autophosphorylationdefinition[GO:0046777]  
2
  • BHF-UCL
Protein phosphorylationdefinition[GO:0006468]  
1
  • UniProtKB
Reciprocal meiotic recombinationdefinition[GO:0007131]  
1
  • PINC
Replicative senescencedefinition[GO:0090399]  
1
  • BHF-UCL
Response to hypoxiadefinition[GO:0001666] silver  
1
  • Ortholog Compara
Response to ionizing radiationdefinition[GO:0010212]  
2
  • UniProtKB
  • BHF-UCL
Signal transductiondefinition[GO:0007165]  
1
  • PINC
Signal transduction involved in mitotic G2 DNA damage checkpointdefinition[GO:0072434]  
1
  • BHF-UCL
Somitogenesisdefinition[GO:0001756] silver  
1
  • Ortholog Compara
Telomere maintenancedefinition[GO:0000723] silver  
1
  • InterPro 2 GO
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 2.7.11.1: ATP + a protein ADP + a phosphoprotein.  
3
  • UniProtKB
It is regulated in the following manner
Inhibited by wortmannin.  
2
  • UniProtKB
Pathways 
According to KEGG, this protein belongs to the following pathways:
Apoptosis  hsa04210+472  
Cell cycle  hsa04110+472  
HTLV-I infection  hsa05166+472  
p53 signaling pathway  hsa04115+472  
According to Reactome, this protein belongs to the following pathways:
ATM mediated phosphorylation of repair proteins  REACT_1924  
ATM mediated response to DNA double-strand break  REACT_204  
Autodegradation of the E3 ubiquitin ligase COP1  REACT_20549  
DNA Damage/Telomere Stress Induced Senescence  REACT_169185  
G2/M DNA damage checkpoint  REACT_897  
Meiotic recombination  REACT_27271  
Recruitment of repair and signaling proteins to double-strand breaks  REACT_97  
Regulation of HSF1-mediated heat shock response  REACT_200780  
Regulation of the Fanconi anemia pathway  REACT_18265  
Stabilization of p53  REACT_309  
Ubiquitin Mediated Degradation of Phosphorylated Cdc25A  REACT_1614  
 

Keywords

Biological process 
Cell cycle  definition   [KW-0131]
DNA damage  definition   [KW-0227]
Molecular function 
Kinase  definition   [KW-0418]
Serine/threonine-protein kinase  definition   [KW-0723]
Transferase  definition   [KW-0808]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

Other
GeneWiki: Ataxia_telangiectasia_mutated
GenomeRNAi: 472
PRO: PR:Q13315
Chemistry
GuidetoPHARMACOLOGY: 1934