ATM acts as a DNA damage sensor upon double strand breaks (DSBs), apoptosis and genotoxic stresses such as ionizing ultraviolet A light (UVA) [, ]. It regulate DNA damage response mechanism through recognising the substrate consensus sequence [ST]-Q and phosphorylates 'Ser-139' of histone variant H2AX/H2AFX at double strand breaks (DSBs) []. On DNA damage, autophosphorylation dissociates ATM into monomers rendering them catalytically active [, ]. It can phosphorylate DYRK2, CHEK2, p53/TP53, FANCD2, NFKBIA, BRCA1, CTIP, nibrin (NBN), TERF1, RAD9 and DCLRE1C [, , , , ]. ATM is also required for telomere elongation []. 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 []. It isalso involved in signal transduction and cell cycle control and may function as a tumour suppressor [].This entry represents the catalytic domain found in serine-protein kinase ATM.
This family consists of serine/threonine protein kinases, including human ATM (Ataxia-Telangiectasia Mutated) and fungal Tel1.ATM acts as a DNA damage sensor upon double strand breaks (DSBs), apoptosis and genotoxic stresses such as ionizing ultraviolet A light (UVA) [, ]. 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) []. On DNA damage, autophosphorylation dissociates ATM into monomers rendering them catalytically active [, ]. It can phosphorylate DYRK2, CHEK2, p53/TP53, FANCD2, NFKBIA, BRCA1, CTIP, nibrin (NBN), TERF1, RAD9 and DCLRE1C [, , , , ]. ATM is also required for telomere elongation []. 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 []. It is also involved in signal transduction and cell cycle control and may function as a tumour suppressor [].
