Serine/threonine-protein kinase ATM (IPR015519)

Short name: ATM

Overlapping homologous superfamilies


Family relationships



This family consists of serine/threonine protein kinases, including human ATM (Ataxia-Telangiectasia Mutated).

ATM acts as a DNA damage sensor upon double strand breaks (DSBs), apoptosis and genotoxic stresses such as ionizing ultraviolet A light (UVA) [PMID: 10973490, PMID: 10873394]. It regulate DNA damage response mechanism through recognizing the substrate consensus sequence [ST]-Q and phosphorylates 'Ser-139' of histone variant H2AX/H2AFX at double strand breaks (DSBs) [PMID: 11571274]. On DNA damage, autophosphorylation dissociates ATM into monomers rendering them catalytically active [PMID: 12556884, PMID: 16858402]. It can phosphorylate DYRK2, CHEK2, p53/TP53, FANCD2, NFKBIA, BRCA1, CTIP, nibrin (NBN), TERF1, RAD9 and DCLRE1C [PMID: 8988033, PMID: 9843217, PMID: 9707615, PMID: 10550055, PMID: 10766245]. ATM is also required for telomere elongation [PMID: 26586427]. ATM 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 [PMID: 19448632]. It is also involved in signal transduction and cell cycle control and may function as a tumour suppressor [PMID: 15680327].

GO terms

Biological Process

GO:0000077 DNA damage checkpoint
GO:0006281 DNA repair
GO:0016572 histone phosphorylation
GO:0090399 replicative senescence
GO:0010212 response to ionizing radiation
GO:0000723 telomere maintenance

Molecular Function

GO:0004674 protein serine/threonine kinase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.