|Group: Atypical Family: PIKK Subfamily: SMG1|
Description: PI-3-kinase-related kinase SMG-1
Gene Symbol: SMG1
Synonyms: 61E3.4, ATX, KIAA0421, LIP
UniprotKB/SwissProt: SMG1_HUMAN (Q96Q15)
Function: Serine/threonine protein kinase involved in both mRNA surveillance and genotoxic stress response pathways. Recognizes the substrate consensus sequence [ST]-Q. Involved in nonsense- mediated decay (NMD) of mRNAs containing premature stop codons by phosphorylating UPF1/RENT1. Also acts as a genotoxic stress- activated protein kinase that displays some functional overlap with ATM. Can phosphorylate TP53/p53 and is required for optimal TP53/p53 activation after cellular exposure to genotoxic stress. Its depletion leads to spontaneous DNA damage and increased sensitivity to ionizing radiation (IR). May activate PRKCI but not PRKCZ.
Subcellular Localization: Nucleus. Cytoplasm.