serine/threonine protein kinase SMG1; SMG-1; hSMG-1; 61E3.4; lambda/iota protein kinase C-interacting protein; lambda-interacting protein (SMG1, ATX, KIAA0421, LIP)

Function: 1) serine/threonine kinase 2) mRNA surveillance 3) genotoxic stress response 4) recognizes the substrate consensus sequence [ST]-Q 5) nonsense-mediated decay (NMD) of mRNAs containing premature stop codons via phosphorylation of UPF1 6) phosphorylates p53 7) depletion leads to spontaneous DNA damage & increased sensitivity to ionizing radiation 8) component of a post-splicing multiprotein NMD complex with UPF1, UPF2, EST1A, EST1B, UPF3 9) component of SMG1C complex 9) autophosphorylated 10) inhibited by caffeine, LY294002, wortmannin Cofactor: Mn+2 Structure: - belongs to the PI3/PI4-kinase family - contains 1 FAT domain - contains 1 FATC domain - contains 1 HEAT repeat - contains 1 PI3K/PI4K domain Compartment: nucleus & cytoplasm Alternative splicing: named isoforms=4 Expression: - widely expressed - expressed in heart, skeletal muscle > placenta, brain, lung, spleen > liver Genetics: - gene is located in a region of chromosome 16 that contains 2 segmental duplications

General

HEAT repeat-containing protein (HEATR) nuclear protein serine/threonine kinase

Properties

SIZE: entity length = 3661 aa MW = 411 kD COMPARTMENT: cytoplasm cell nucleus MOTIF: SMG8/SMG9 interaction {1-1977} MOTIF: FAT domain NAME: FAT domain SITE: 1131-1866 MOTIF: Tyr phosphorylation site binding site FOR-BINDING-OF: Paxillin HEAT repeat {1817-1852} PI3K/PI4K {2150-2478} Thr phosphorylation site {T3550} Ser phosphorylation site {S3556} Ser phosphorylation site {S3570} Thr phosphorylation site {T3573} Ser phosphorylation site {S3576} Thr phosphorylation site {T3577} FATC {3629-3661} STATE: active state

Database Correlations

OMIM 607032 UniProt Q96Q15 PFAM correlations ENZYME 2.7.11.1

References

UniProt :accession Q96Q15