ATM Products

Antibodies
ATM Antibody (2C1)
ATM Antibody (2C1)
Species: Hu, Mu, Rt, Pm
Applications: WB, ELISA, Flow, ICC/IF, IHC, IP, PAGE, ChIP, KD, Single-Cell Western
Host: Mouse Monoclonal
ELISA Kits
Human ATM ELISA Kit (Colorime ...
Human ATM ELISA Kit (Colorimetric)
NBP2-69891
Species: Hu
Applications: ELISA
Rat ATM ELISA Kit (Colorimetr ...
Rat ATM ELISA Kit (Colorimetric)
NBP2-69893
Species: Rt
Applications: ELISA
Mouse ATM ELISA Kit (Colorime ...
Mouse ATM ELISA Kit (Colorimetric)
NBP2-69892
Species: Mu
Applications: ELISA
Proteins
Recombinant Human ATM GST (N- ...
Recombinant Human ATM GST (N-Term)...
H00000472-Q01
Species: Hu
Applications: WB, ELISA, MA, PAGE, AP
Recombinant Human ATM GST (N- ...
Recombinant Human ATM GST (N-Term)...
H00000472-P01
Species: Hu
Applications: WB, ELISA, MA, PAGE, AP
ATM Recombinant Protein Antig ...
ATM Recombinant Protein Antigen
NBP2-57867PEP
Species: Hu
Applications: AC

Description

ATM (ataxia telangiectasia mutated kinase) is the master regulator of the DNA double-strand break (DSB) repair pathway. This ubiquitously expressed serine/threonine protein kinase belongs to the PI3K-like family of proteins and responds to DSBs caused by oxidative and other genotoxic stresses (1). In addition to regulating the DNA damage response, ATM participates in vesicle and protein transport, T-cell development, gonads/neurological function, pre-B cell allelic exclusion, cell cycle control, and acts as a tumor suppressor (2,3). Defects in ATM are associated with ataxia telangiectasia (AT), T-cell acute lymphoblastic leukemia (TALL), T-prolymphocytic leukemia (TPLL), and B-cell non-Hodgkin lymphomas (BNHL) including mantle cell lymphoma (MCL) and B-cell chronic lymphocytic leukemia (BCLL) (4).

The theoretical molecular weight of ATM is 350 kDa and it has 3 main domains: a FAT (focal adhesion targeting) domain (aa 1960-2566), a PI-3/PI-4 kinase catalytic domain (aa 2712-2962), and a C-terminal FAT domain (aa 3024-3056). ATM exists as a dimer or tetramer in its inactive state. Upon sensing DNA damage, the MRE11-RAD50-NBS1 (MRN) complex recruits ATM. The intricate process of ATM activation involves acetylation by KAT5/TIP60, autophosphorylation at Ser-1981, and dissociation into catalytically active monomers (5). Following activation, ATM phosphorylates multiple substrates such as p53/TP53 and Chk2 involved in DNA repair, checkpoint signaling, and the apoptosis pathway.

References

1. Paull TT. (2015) Mechanisms of ATM Activation. Annu Rev Biochem. 84:711-38. PMID: 25580527

2. Chaudhary MW and Al-Baradie RS. (2014) Ataxia-telangiectasia: future prospects. Appl Clin Genet. 7:159-167. PMID: 25258552

3. Stagni V, Cirotti C, and Barila D. (2018) Ataxia-Telangiectasia Mutated Kinase in the Control of Oxidative Stress, Mitochondria, and Autophagy in Cancer: A Maestro With a Large Orchestra. Front Oncol. 8:73. PMID: 29616191

4. Gumy-Pause F, Wacker P, and Sappino AP. (2004) ATM gene and lymphoid malignancies. Leukemia. 18(2):238-42. PMID: 14628072

5. Adamowicz M. (2018) Breaking up with ATM. J Immunol Sci. 2(1):26-31. PMID: 29652413



Bioinformatics

Entrez Mouse
Rat
Human
Uniprot Human
Human
Human
Human
Alternate Names
  • AT mutated
  • A-T mutated
  • AT1
  • ATA
  • ataxia telangiectasia mutated (includes complementation groups A, C and D)
  • ataxia telangiectasia mutated
  • ATC
  • ATD
  • ATDC
  • ATE
  • ATM serine/threonine kinase
  • DKFZp781A0353
  • EC 2.7.11.1
  • MGC74674
  • serine-protein kinase ATM
  • TEL1
  • TEL1, telomere maintenance 1, homolog
  • TELO1