Regulatory Network in Protein Phosphorylation
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Kinase: ATM ataxia telangiectasia mutated (includes complementation groups A, C and D)

Group: Atypical      Family: PIKK      Subfamily: ATM

Description: ataxia telangiectasia mutated (includes complementation groups A, C and D)

Gene Symbol: ATM

Synonyms: AT1, ATA, ATC, ATD, ATDC, ATE, DKFZp781A0353, MGC74674, TEL1, TELO1

UniprotKB/SwissProt: ATM_HUMAN (Q13315)

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.

Subcellular Localization: Nucleus. Cytoplasmic vesicle. Note=Primarily nuclear. Found also in endocytic vesicles in association with beta-adaptin.

Protein Domain:

The substrate proteins of kinase ATM

No.Gene NameUniProtKB IDProtein DescriptionNumber of kinase-specific phosphorylation sitesView
1EIF4EBP14EBP1_HUMANEukaryotic translation initiation factor 4E-binding protein 1 (4E-BP1)(eIF4E-binding protein 1) (Phosphorylated heat- and acid-stableprotein regulated by insulin 1) (PHAS-I). 3Show
2ABL1ABL1_HUMANProto-oncogene tyrosine-protein kinase ABL1 (EC 2.7.10.2) (p150) (c-ABL) (Abelson murine leukemia viral oncogene homolog 1). 2Show
3ATF2ATF2_HUMANCyclic AMP-dependent transcription factor ATF-2 (Activatingtranscription factor 2) (cAMP response element-binding protein CRE-BP1) (HB16). 2Show
4ATMATM_HUMANSerine-protein kinase ATM (EC 2.7.11.1) (Ataxia telangiectasiamutated) (A-T, mutated). 5Show
5BLMBLM_HUMANBloom syndrome protein (EC 3.6.1.-) (RecQ protein-like 3) (DNAhelicase, RecQ-like type 2). 1Show
6BRCA1BRCA1_HUMANBreast cancer type 1 susceptibility protein (RING finger protein 53). 24Show
7CHEK1CHK1_HUMANSerine/threonine-protein kinase Chk1 (EC 2.7.11.1). 5Show
8CHEK2CHK2_HUMANSerine/threonine-protein kinase Chk2 (EC 2.7.11.1) (Cds1). 16Show
9CREB1CREB1_HUMANcAMP response element-binding protein (CREB). 2Show
10DCLRE1CDCR1C_HUMANArtemis protein (EC 3.1.-.-) (DNA cross-link repair 1C protein) (SNM1-like protein) (A-SCID protein) (hSNM1C). 10Show
11LIG4DNL4_HUMANDNA ligase 4 (EC 6.5.1.1) (DNA ligase IV) (Polydeoxyribonucleotidesynthase [ATP] 4). 1Show
12E2F1E2F1_HUMANTranscription factor E2F1 (E2F-1) (Retinoblastoma-binding protein 3)(RBBP-3) (PRB-binding protein E2F-1) (PBR3) (Retinoblastoma-associatedprotein 1) (RBAP-1). 2Show
13FANCD2FACD2_HUMANFanconi anemia group D2 protein (Protein FACD2). 7Show
14H2AFXH2AX_HUMANHistone H2A.x (H2a/x). 3Show
15MCM3MCM3_HUMANDNA replication licensing factor MCM3 (DNA polymerase alphaholoenzyme-associated protein P1) (RLF subunit beta) (P102 protein)(P1-MCM3). 1Show
16MDM2MDM2_HUMANE3 ubiquitin-protein ligase Mdm2 (EC 6.3.2.-) (p53-binding proteinMdm2) (Oncoprotein Mdm2) (Double minute 2 protein) (Hdm2). 2Show
17MRE11AMRE11_HUMANDouble-strand break repair protein MRE11A (MRE11 homolog 1) (MRE11meiotic recombination 11 homolog A). 2Show
18NBNNBN_HUMANNibrin (Nijmegen breakage syndrome protein 1) (Cell cycle regulatoryprotein p95). 8Show
19IKBKGNEMO_HUMANNF-kappa-B essential modulator (NEMO) (NF-kappa-B essential modifier)(Inhibitor of nuclear factor kappa-B kinase subunit gamma) (IkB kinasesubunit gamma) (I-kappa-B kinase gamma) (IKK-gamma) (IKKG) (IkBkinase-associated protein 1) (IKKAP1) (FIP-3). 1Show
20NFAT5NFAT5_HUMANNuclear factor of activated T-cells 5 (T-cell transcription factorNFAT5) (NF-AT5) (Tonicity-responsive enhancer-binding protein) (TonE-binding protein) (TonEBP). 3Show
21TP53P53_HUMANCellular tumor antigen p53 (Tumor suppressor p53) (Phosphoprotein p53)(Antigen NY-CO-13). 7Show
22RAD17RAD17_HUMANCell cycle checkpoint protein RAD17 (hRad17) (RF-C/activator 1homolog). 7Show
23RAD50RAD50_HUMANDNA repair protein RAD50 (EC 3.6.-.-) (hRAD50). 1Show
24RAD9ARAD9A_HUMANCell cycle checkpoint control protein RAD9A (EC 3.1.11.2) (DNA repairexonuclease rad9 homolog A) (hRAD9). 2Show
25RBBP8RBBP8_HUMANRetinoblastoma-binding protein 8 (RBBP-8) (CtBP-interacting protein)(CtIP) (Retinoblastoma-interacting protein and myosin-like) (RIM). 8Show
26RPA2RFA2_HUMANReplication protein A 32 kDa subunit (RP-A) (RF-A) (Replicationfactor-A protein 2) (p32) (p34). 1Show
27SMC1ASMC1A_HUMANStructural maintenance of chromosomes protein 1A (SMC1alpha protein)(Sb1.8). 6Show
28STK11STK11_HUMANSerine/threonine-protein kinase 11 (EC 2.7.11.1) (Serine/threonine-protein kinase LKB1) (Renal carcinoma antigen NY-REN-19). 2Show
29TBL2TBL2_HUMANTransducin beta-like 2 protein (WS beta-transducin repeats protein)(WS-betaTRP) (Williams-Beuren syndrome chromosomal region 13 protein). 1Show
30TERF1TERF1_HUMANTelomeric repeat-binding factor 1 (TTAGGG repeat-binding factor 1)(NIMA-interacting protein 2) (Telomeric protein Pin2/TRF1). 3Show
31TOPBP1TOPB1_HUMANDNA topoisomerase 2-binding protein 1 (DNA topoisomerase II-bindingprotein 1) (DNA topoisomerase IIbeta-binding protein 1) (TopBP1). 5Show
32TP53BP1TP53B_HUMANTumor suppressor p53-binding protein 1 (p53-binding protein 1)(p53BP1) (53BP1). 8Show
33WRNWRN_HUMANWerner syndrome ATP-dependent helicase (EC 3.6.1.-). 4Show
34XPAXPA_HUMANDNA-repair protein complementing XP-A cells (Xeroderma pigmentosumgroup A-complementing protein). 2Show



The substrate proteins of kinase family ATM

No.Gene NameUniProtKB IDProtein DescriptionNumber of kinase family-specific phosphorylated sitesView
1EIF4EBP14EBP1_HUMANEukaryotic translation initiation factor 4E-binding protein 1 (4E-BP1)(eIF4E-binding protein 1) (Phosphorylated heat- and acid-stableprotein regulated by insulin 1) (PHAS-I). 3Show
2ABL1ABL1_HUMANProto-oncogene tyrosine-protein kinase ABL1 (EC 2.7.10.2) (p150) (c-ABL) (Abelson murine leukemia viral oncogene homolog 1). 2Show
3ATF2ATF2_HUMANCyclic AMP-dependent transcription factor ATF-2 (Activatingtranscription factor 2) (cAMP response element-binding protein CRE-BP1) (HB16). 2Show
4ATMATM_HUMANSerine-protein kinase ATM (EC 2.7.11.1) (Ataxia telangiectasiamutated) (A-T, mutated). 5Show
5BLMBLM_HUMANBloom syndrome protein (EC 3.6.1.-) (RecQ protein-like 3) (DNAhelicase, RecQ-like type 2). 1Show
6BRCA1BRCA1_HUMANBreast cancer type 1 susceptibility protein (RING finger protein 53). 24Show
7CHEK1CHK1_HUMANSerine/threonine-protein kinase Chk1 (EC 2.7.11.1). 5Show
8CHEK2CHK2_HUMANSerine/threonine-protein kinase Chk2 (EC 2.7.11.1) (Cds1). 16Show
9CREB1CREB1_HUMANcAMP response element-binding protein (CREB). 2Show
10DCLRE1CDCR1C_HUMANArtemis protein (EC 3.1.-.-) (DNA cross-link repair 1C protein) (SNM1-like protein) (A-SCID protein) (hSNM1C). 10Show
11LIG4DNL4_HUMANDNA ligase 4 (EC 6.5.1.1) (DNA ligase IV) (Polydeoxyribonucleotidesynthase [ATP] 4). 1Show
12E2F1E2F1_HUMANTranscription factor E2F1 (E2F-1) (Retinoblastoma-binding protein 3)(RBBP-3) (PRB-binding protein E2F-1) (PBR3) (Retinoblastoma-associatedprotein 1) (RBAP-1). 2Show
13FANCD2FACD2_HUMANFanconi anemia group D2 protein (Protein FACD2). 7Show
14H2AFXH2AX_HUMANHistone H2A.x (H2a/x). 3Show
15MCM3MCM3_HUMANDNA replication licensing factor MCM3 (DNA polymerase alphaholoenzyme-associated protein P1) (RLF subunit beta) (P102 protein)(P1-MCM3). 1Show
16MDM2MDM2_HUMANE3 ubiquitin-protein ligase Mdm2 (EC 6.3.2.-) (p53-binding proteinMdm2) (Oncoprotein Mdm2) (Double minute 2 protein) (Hdm2). 2Show
17MRE11AMRE11_HUMANDouble-strand break repair protein MRE11A (MRE11 homolog 1) (MRE11meiotic recombination 11 homolog A). 2Show
18NBNNBN_HUMANNibrin (Nijmegen breakage syndrome protein 1) (Cell cycle regulatoryprotein p95). 8Show
19IKBKGNEMO_HUMANNF-kappa-B essential modulator (NEMO) (NF-kappa-B essential modifier)(Inhibitor of nuclear factor kappa-B kinase subunit gamma) (IkB kinasesubunit gamma) (I-kappa-B kinase gamma) (IKK-gamma) (IKKG) (IkBkinase-associated protein 1) (IKKAP1) (FIP-3). 1Show
20NFAT5NFAT5_HUMANNuclear factor of activated T-cells 5 (T-cell transcription factorNFAT5) (NF-AT5) (Tonicity-responsive enhancer-binding protein) (TonE-binding protein) (TonEBP). 3Show
21TP53P53_HUMANCellular tumor antigen p53 (Tumor suppressor p53) (Phosphoprotein p53)(Antigen NY-CO-13). 7Show
22RAD17RAD17_HUMANCell cycle checkpoint protein RAD17 (hRad17) (RF-C/activator 1homolog). 7Show
23RAD50RAD50_HUMANDNA repair protein RAD50 (EC 3.6.-.-) (hRAD50). 1Show
24RAD9ARAD9A_HUMANCell cycle checkpoint control protein RAD9A (EC 3.1.11.2) (DNA repairexonuclease rad9 homolog A) (hRAD9). 2Show
25RBBP8RBBP8_HUMANRetinoblastoma-binding protein 8 (RBBP-8) (CtBP-interacting protein)(CtIP) (Retinoblastoma-interacting protein and myosin-like) (RIM). 8Show
26RPA2RFA2_HUMANReplication protein A 32 kDa subunit (RP-A) (RF-A) (Replicationfactor-A protein 2) (p32) (p34). 1Show
27SMC1ASMC1A_HUMANStructural maintenance of chromosomes protein 1A (SMC1alpha protein)(Sb1.8). 6Show
28STK11STK11_HUMANSerine/threonine-protein kinase 11 (EC 2.7.11.1) (Serine/threonine-protein kinase LKB1) (Renal carcinoma antigen NY-REN-19). 2Show
29TBL2TBL2_HUMANTransducin beta-like 2 protein (WS beta-transducin repeats protein)(WS-betaTRP) (Williams-Beuren syndrome chromosomal region 13 protein). 1Show
30TERF1TERF1_HUMANTelomeric repeat-binding factor 1 (TTAGGG repeat-binding factor 1)(NIMA-interacting protein 2) (Telomeric protein Pin2/TRF1). 3Show
31TOPBP1TOPB1_HUMANDNA topoisomerase 2-binding protein 1 (DNA topoisomerase II-bindingprotein 1) (DNA topoisomerase IIbeta-binding protein 1) (TopBP1). 5Show
32TP53BP1TP53B_HUMANTumor suppressor p53-binding protein 1 (p53-binding protein 1)(p53BP1) (53BP1). 8Show
33WRNWRN_HUMANWerner syndrome ATP-dependent helicase (EC 3.6.1.-). 4Show
34XPAXPA_HUMANDNA-repair protein complementing XP-A cells (Xeroderma pigmentosumgroup A-complementing protein). 2Show