ATM Products

Antibodies
ATM Antibody (2C1)
ATM Antibody (2C1)
Species: Hu, Mu, Rt, Pm
Applications: WB, ChIP, ELISA, Flow, ICC/IF, IHC, IHC-P, IP, KD
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, PA, PAGE, AP
Recombinant Human ATM GST (N- ...
Recombinant Human ATM GST (N-Term)...
H00000472-P01
Species: Hu
Applications: WB, ELISA, PA, 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 Human
Mouse
Rat
Uniprot Human
Human
Human
Human
Product By Gene ID 472
Alternate Names
  • ataxia telangiectasia mutated
  • ATD
  • ATM serine/threonine kinase
  • ATC
  • TEL1, telomere maintenance 1, homolog
  • ataxia telangiectasia mutated (includes complementation groups A, C and D)
  • serine-protein kinase ATM
  • ATA
  • AT mutated
  • AT1
  • MGC74674
  • EC 2.7.11.1
  • DKFZp781A0353
  • A-T mutated
  • ATE
  • TELO1
  • ATDC
  • TEL1

Bioinformatics Tool for ATM

Discover related pathways, diseases and genes to ATM. Need help? Read the Bioinformatics Tool Guide for instructions on using this tool.
 
Vizit™, under license from BioVista Inc.

Related ATM Blog Posts

Check out the latest blog posts on ATM.
Further unraveling the role of gamma H2AX in DNA damage response
Our genome experiences a moderate amount of DNA damage in our cells on a daily basis.  This DNA damage can be in response to external environmental factors, or be a result of our internal metabolic processes going awry.  While normal rates of DNA ...    Read more.
The recent relationship of BRCA1 and 53BP1
The p53-binding protein 1 (53BP1) is a DNA damage response factor, which is recruited to nuclear structures at the site of DNA damage.  DNA double-strand breaks (DSBs) are mutations that are detrimental to cell viability and genome stability, and m...    Read more.
Application Highlight: Recent uses of TERF2 in immunofluorescence (IF)
Telomeres are a region of repeat nucleotide sequences located at the end of chromosomes to protect our DNA from becoming damaged via end-to-end fusion.  TERF2, or telomeric-repeat binding factor 2, is important for telomere integrity and aids in th...    Read more.
ATM - detecting and responding to DNA damage
Ataxia telangiectasia mutated (ATM) is essential for the maintenance of genomic stability. ATM is a 370 kDa serine-threonine kinase that is constitutively expressed in various tissues. Although primarily nuclear, ATM is also found at lower levels...    Read more.
53BP1 - a marker for DNA Double Strand Break
53BP1 (p53 binding protein 1) was originally thought to be an enhancer for p53 transcriptional, but later studies have demonstrated that it is actually a substrate for ataxia telangiectasia mutated (ATM). 53BP1 is a classic late DNA damage response...    Read more.
53BP1 - DNA damage is no fun
The 53BP1 (p53 binding protein 1) was initially believed to be a p53 transcriptional enhancing partner, but it has now been established as an ataxia telangiectasia mutated (ATM) substrate. As a late DNA damage response (DDR) marker, 53BP1 appears duri...    Read more.
ATM and DSB Repair in Cancer
Ataxia Telangiectasia Mutated (ATM) is a serine/threonine protein kinase that is the master regulator of the DNA double-strand break (DSB) repair pathway. ATM is a key part of the cell cycle machinery that activates checkpoint signaling in response to...    Read more.
Read more ATM related blogs.