UPF1

Summary

Gene Symbol: UPF1
Description: UPF1, RNA helicase and ATPase
Alias: HUPF1, NORF1, RENT1, pNORF1, smg-2, regulator of nonsense transcripts 1, ATP-dependent helicase RENT1, UPF1 regulator of nonsense transcripts homolog, delta helicase, nonsense mRNA reducing factor 1, smg-2 homolog, nonsense mediated mRNA decay factor, up-frameshift mutation 1 homolog, up-frameshift suppressor 1 homolog, yeast Upf1p homolog
Species: human
Products:     UPF1

Top Publications

  1. Yamashita A, Ohnishi T, Kashima I, Taya Y, Ohno S. Human SMG-1, a novel phosphatidylinositol 3-kinase-related protein kinase, associates with components of the mRNA surveillance complex and is involved in the regulation of nonsense-mediated mRNA decay. Genes Dev. 2001;15:2215-28 pubmed
    ..hSMG-1 phosphorylates hUPF1/SMG-2 in vivo and in vitro at specific serine residues in SQ motifs...
  2. Clerici M, Mourão A, Gutsche I, Gehring N, Hentze M, Kulozik A, et al. Unusual bipartite mode of interaction between the nonsense-mediated decay factors, UPF1 and UPF2. EMBO J. 2009;28:2293-306 pubmed publisher
    ..In mammalian cells, NMD is triggered when UPF2 bound to UPF3 on a downstream exon junction complex interacts with UPF1 bound to a stalled ribosome...
  3. Fiorini F, Boudvillain M, Le Hir H. Tight intramolecular regulation of the human Upf1 helicase by its N- and C-terminal domains. Nucleic Acids Res. 2013;41:2404-15 pubmed publisher
    The RNA helicase Upf1 is a multifaceted eukaryotic enzyme involved in DNA replication, telomere metabolism and several mRNA degradation pathways...
  4. Czaplinski K, Ruiz Echevarria M, Paushkin S, Han X, Weng Y, Perlick H, et al. The surveillance complex interacts with the translation release factors to enhance termination and degrade aberrant mRNAs. Genes Dev. 1998;12:1665-77 pubmed
    ..The product of the UPF1 gene is a necessary component of the putative surveillance complex that recognizes and degrades aberrant mRNAs...
  5. Cho H, Kim K, Kim Y. Human proline-rich nuclear receptor coregulatory protein 2 mediates an interaction between mRNA surveillance machinery and decapping complex. Mol Cell. 2009;33:75-86 pubmed publisher
    ..Here we identify human proline-rich nuclear receptor coregulatory protein 2 (PNRC2) as a Upf1- and Dcp1a-interacting protein...
  6. Chiu S, Serin G, Ohara O, Maquat L. Characterization of human Smg5/7a: a protein with similarities to Caenorhabditis elegans SMG5 and SMG7 that functions in the dephosphorylation of Upf1. RNA. 2003;9:77-87 pubmed
    Nonsense-mediated mRNA decay (NMD) in mammalian cells depends on phosphorylation of Upf1, an RNA-dependent ATPase and 5'-to-3' helicase. Upf1 phosphorylation is mediated by Smg1, a phosphoinositol 3-kinase-related protein kinase...
  7. Lykke Andersen J, Shu M, Steitz J. Human Upf proteins target an mRNA for nonsense-mediated decay when bound downstream of a termination codon. Cell. 2000;103:1121-31 pubmed
    ..While in HeLa cell extracts these proteins are complexed with hUpf1, in intact cells hUpf3a and hUpf3b are nucleocytoplasmic shuttling proteins, hUpf2 is perinuclear, and hUpf1 ..
  8. Applequist S, Selg M, Raman C, Jäck H. Cloning and characterization of HUPF1, a human homolog of the Saccharomyces cerevisiae nonsense mRNA-reducing UPF1 protein. Nucleic Acids Res. 1997;25:814-21 pubmed
    ..We have thus identified the first mammalian homolog of yeast UPF1, a protein that regulates levels of nonsense mRNA, and we tentatively name this protein human HUPF1 (for human homolog of UPF1).
  9. Chan W, Bhalla A, Le Hir H, Nguyen L, Huang L, Gecz J, et al. A UPF3-mediated regulatory switch that maintains RNA surveillance. Nat Struct Mol Biol. 2009;16:747-53 pubmed publisher
    ..Together, our results suggest that UPF3A levels are tightly regulated by a post-transcriptional switch to maintain appropriate levels of NMD substrates in cells containing different levels of UPF3B. ..

More Information

Publications77

  1. Kaygun H, Marzluff W. Regulated degradation of replication-dependent histone mRNAs requires both ATR and Upf1. Nat Struct Mol Biol. 2005;12:794-800 pubmed
    ..Here we show that regulated degradation of histone mRNAs requires Upf1, a key regulator of the nonsense-mediated decay pathway, and ATR, a key regulator of the DNA damage checkpoint ..
  2. Melero R, Buchwald G, Castaño R, Raabe M, Gil D, Lázaro M, et al. The cryo-EM structure of the UPF-EJC complex shows UPF1 poised toward the RNA 3' end. Nat Struct Mol Biol. 2012;19:498-505, S1-2 pubmed publisher
    ..assembly is built around UPF2, a scaffold protein with a ring structure that closes around the CH domain of UPF1, keeping the helicase region in an accessible and unwinding-competent state...
  3. Jonas S, Weichenrieder O, Izaurralde E. An unusual arrangement of two 14-3-3-like domains in the SMG5-SMG7 heterodimer is required for efficient nonsense-mediated mRNA decay. Genes Dev. 2013;27:211-25 pubmed publisher
    ..are recruited to PTC-containing mRNAs through the interaction of their 14-3-3-like domains with phosphorylated UPF1, the central NMD effector. Recruitment of SMG5, SMG6, and SMG7 causes NMD target degradation...
  4. Page M, Carr B, Anders K, Grimson A, Anderson P. SMG-2 is a phosphorylated protein required for mRNA surveillance in Caenorhabditis elegans and related to Upf1p of yeast. Mol Cell Biol. 1999;19:5943-51 pubmed
    ..smg-2 is homologous to UPF1 of yeast and to RENT1 (also called HUPF1), a human gene likely involved in NMD...
  5. Denning G, Jamieson L, Maquat L, Thompson E, Fields A. Cloning of a novel phosphatidylinositol kinase-related kinase: characterization of the human SMG-1 RNA surveillance protein. J Biol Chem. 2001;276:22709-14 pubmed
    ..hSMG-1 phosphorylates purified hUpf1 protein, a phosphoprotein that plays a critical role in NMD, at sites that are also phosphorylated in whole cells...
  6. Singh G, Rebbapragada I, Lykke Andersen J. A competition between stimulators and antagonists of Upf complex recruitment governs human nonsense-mediated mRNA decay. PLoS Biol. 2008;6:e111 pubmed publisher
    ..The conserved Upf1-3 complex interacts with the eukaryotic translation release factors, eRF3 and eRF1, and triggers NMD when ..
  7. Hwang J, Sato H, Tang Y, Matsuda D, Maquat L. UPF1 association with the cap-binding protein, CBP80, promotes nonsense-mediated mRNA decay at two distinct steps. Mol Cell. 2010;39:396-409 pubmed publisher
    ..Here we show that precluding the binding of the NMD factor UPF1 to CBP80 inhibits NMD at two steps: the association of SMG1 and UPF1 with the two eukaryotic release factors (eRFs) ..
  8. Isken O, Kim Y, Hosoda N, Mayeur G, Hershey J, Maquat L. Upf1 phosphorylation triggers translational repression during nonsense-mediated mRNA decay. Cell. 2008;133:314-27 pubmed publisher
    ..The subsequent binding of Upf1 to the EJC triggers Upf1 phosphorylation...
  9. Azzalin C, Lingner J. The human RNA surveillance factor UPF1 is required for S phase progression and genome stability. Curr Biol. 2006;16:433-9 pubmed
    ..In humans, NMD depends on the RNA- and DNA-dependent 5'-3' helicase UPF1 and six other gene products referred to as SMG1, UPF2, UPF3, EST1A/SMG6, EST1B/SMG5, and EST1C/SMG7...
  10. Mendell J, ap Rhys C, Dietz H. Separable roles for rent1/hUpf1 in altered splicing and decay of nonsense transcripts. Science. 2002;298:419-22 pubmed
    ..Inhibition of rent1/hUpf1 expression abrogated both NMD and NAS of nonsense T cell receptor beta transcripts...
  11. Zünd D, Gruber A, Zavolan M, Mühlemann O. Translation-dependent displacement of UPF1 from coding sequences causes its enrichment in 3' UTRs. Nat Struct Mol Biol. 2013;20:936-43 pubmed publisher
    Recruitment of the UPF1 nonsense-mediated mRNA decay (NMD) factor to target mRNAs was initially proposed to occur through interaction with release factors at terminating ribosomes...
  12. Kashima I, Yamashita A, Izumi N, Kataoka N, Morishita R, Hoshino S, et al. Binding of a novel SMG-1-Upf1-eRF1-eRF3 complex (SURF) to the exon junction complex triggers Upf1 phosphorylation and nonsense-mediated mRNA decay. Genes Dev. 2006;20:355-67 pubmed
    ..While it is known that a key event in the triggering of NMD is phosphorylation of the trans-acting factor, Upf1, by SMG-1, the relationship between Upf1 phosphorylation and PTC recognition remains undetermined...
  13. Azzalin C, Lingner J. The double life of UPF1 in RNA and DNA stability pathways. Cell Cycle. 2006;5:1496-8 pubmed
    The DNA and RNA helicase UPF1 is well known for its central role in Nonsense Mediated RNA Decay (NMD), which promotes degradation of mRNAs containing premature stop codons...
  14. Kim Y, Furic L, DesGroseillers L, Maquat L. Mammalian Staufen1 recruits Upf1 to specific mRNA 3'UTRs so as to elicit mRNA decay. Cell. 2005;120:195-208 pubmed
    ..Here we describe an mRNA decay mechanism that involves Stau1, the NMD factor Upf1, and a termination codon...
  15. Fukuhara N, Ebert J, Unterholzner L, Lindner D, Izaurralde E, Conti E. SMG7 is a 14-3-3-like adaptor in the nonsense-mediated mRNA decay pathway. Mol Cell. 2005;17:537-47 pubmed
    In metazoa, regulation of the phosphorylation state of UPF1 is crucial for nonsense-mediated mRNA decay (NMD), a process by which aberrant mRNAs containing nonsense mutations are degraded...
  16. Cheng Z, Muhlrad D, Lim M, Parker R, Song H. Structural and functional insights into the human Upf1 helicase core. EMBO J. 2007;26:253-64 pubmed
    ..These conformational changes alter the likely ssRNA-binding channel in a manner that can explain how ATP binding destabilizes ssRNA binding to Upf1p...
  17. Ivanov P, Gehring N, Kunz J, Hentze M, Kulozik A. Interactions between UPF1, eRFs, PABP and the exon junction complex suggest an integrated model for mammalian NMD pathways. EMBO J. 2008;27:736-47 pubmed publisher
    ..Phosphorylation of the protein UPF1 in the context of translation termination contributes to committing mRNAs to NMD...
  18. Chakrabarti S, Jayachandran U, Bonneau F, Fiorini F, Basquin C, Domcke S, et al. Molecular mechanisms for the RNA-dependent ATPase activity of Upf1 and its regulation by Upf2. Mol Cell. 2011;41:693-703 pubmed publisher
    b>Upf1 is a crucial factor in nonsense-mediated mRNA decay, the eukaryotic surveillance pathway that degrades mRNAs containing premature stop codons...
  19. Chawla R, Redon S, Raftopoulou C, Wischnewski H, Gagos S, Azzalin C. Human UPF1 interacts with TPP1 and telomerase and sustains telomere leading-strand replication. EMBO J. 2011;30:4047-58 pubmed publisher
    Eukaryotic up-frameshift 1 (UPF1) is a nucleic acid-dependent ATPase and 5'-to-3' helicase, best characterized for its roles in cytoplasmic RNA quality control...
  20. Bhattacharya A, Czaplinski K, Trifillis P, He F, Jacobson A, Peltz S. Characterization of the biochemical properties of the human Upf1 gene product that is involved in nonsense-mediated mRNA decay. RNA. 2000;6:1226-35 pubmed
    ..A human homolog of the yeast UPF1, termed HUpf1/RENT1, has also been identified. The HUpf1 has also been shown to play a role in NMD in mammalian cells...
  21. Hogg J, Goff S. Upf1 senses 3'UTR length to potentiate mRNA decay. Cell. 2010;143:379-89 pubmed publisher
    ..assembled on transcripts with HIV-1 3' untranslated region (3'UTR) sequences, we uncover a two-step mechanism for Upf1-dependent degradation of mRNAs with long 3'UTRs...
  22. Karam R, Carvalho J, Bruno I, Graziadio C, Senz J, Huntsman D, et al. The NMD mRNA surveillance pathway downregulates aberrant E-cadherin transcripts in gastric cancer cells and in CDH1 mutation carriers. Oncogene. 2008;27:4255-60 pubmed publisher
    ..tumour cell line were upregulated in response to protein synthesis inhibitors or depletion of the NMD factors UPF1 and eIF4AIII...
  23. Yamashita A, Izumi N, Kashima I, Ohnishi T, Saari B, Katsuhata Y, et al. SMG-8 and SMG-9, two novel subunits of the SMG-1 complex, regulate remodeling of the mRNA surveillance complex during nonsense-mediated mRNA decay. Genes Dev. 2009;23:1091-105 pubmed publisher
    ..SMG-1 and Upf1 transiently form a surveillance complex termed "SURF" that includes eRF1 and eRF3 on post-spliced mRNAs ..
  24. Singh G, Jakob S, Kleedehn M, Lykke Andersen J. Communication with the exon-junction complex and activation of nonsense-mediated decay by human Upf proteins occur in the cytoplasm. Mol Cell. 2007;27:780-92 pubmed
    ..Furthermore, retention of the NMD factor hUpf1 in the nucleus strongly impairs NMD...
  25. You K, Li L, Kim N, Kang H, Koh K, Chwae Y, et al. Selective translational repression of truncated proteins from frameshift mutation-derived mRNAs in tumors. PLoS Biol. 2007;5:e109 pubmed
    ..Our findings suggest a novel mechanism of gene expression regulation for PTC-containing mRNAs in which the deleterious transcripts are regulated either by NMD or translational repression. ..
  26. Kadlec J, Guilligay D, Ravelli R, Cusack S. Crystal structure of the UPF2-interacting domain of nonsense-mediated mRNA decay factor UPF1. RNA. 2006;12:1817-24 pubmed
    b>UPF1 is an essential eukaryotic RNA helicase that plays a key role in various mRNA degradation pathways, notably nonsense-mediated mRNA decay (NMD)...
  27. Brumbaugh K, Otterness D, Geisen C, Oliveira V, Brognard J, Li X, et al. The mRNA surveillance protein hSMG-1 functions in genotoxic stress response pathways in mammalian cells. Mol Cell. 2004;14:585-98 pubmed
    ..target sequences in the checkpoint protein p53 and the nonsense-mediated mRNA decay (NMD) protein hUpf1. Expression of hSMG-1 is required for optimal p53 activation after cellular exposure to genotoxic stress, and ..
  28. Ajamian L, Abrahamyan L, Milev M, Ivanov P, Kulozik A, Gehring N, et al. Unexpected roles for UPF1 in HIV-1 RNA metabolism and translation. RNA. 2008;14:914-27 pubmed publisher
    ..In this report, we show that the nonsense-mediated decay (NMD) factor UPF1 is also a component of the HIV-1 RNP. We investigated the role of UPF1 in HIV-1-expressing cells...
  29. Lykke Andersen J, Shu M, Steitz J. Communication of the position of exon-exon junctions to the mRNA surveillance machinery by the protein RNPS1. Science. 2001;293:1836-9 pubmed
    ..Significantly, RNPS1 triggers NMD when tethered to the 3' untranslated region of beta-globin mRNA, demonstrating its role as a subunit of the postsplicing complex directly involved in mRNA surveillance...
  30. Mocquet V, Neusiedler J, Rende F, Cluet D, Robin J, Terme J, et al. The human T-lymphotropic virus type 1 tax protein inhibits nonsense-mediated mRNA decay by interacting with INT6/EIF3E and UPF1. J Virol. 2012;86:7530-43 pubmed publisher
    ..factor 3 (eIF3) required for efficient NMD, as well as the NMD core factor upstream frameshift protein 1 (UPF1)...
  31. Ohnishi T, Yamashita A, Kashima I, Schell T, Anders K, Grimson A, et al. Phosphorylation of hUPF1 induces formation of mRNA surveillance complexes containing hSMG-5 and hSMG-7. Mol Cell. 2003;12:1187-200 pubmed
    ..Here we show that phosphorylated hUPF1, the human ortholog of UPF1/SMG-2, forms a complex with human orthologs of the C...
  32. Franks T, Singh G, Lykke Andersen J. Upf1 ATPase-dependent mRNP disassembly is required for completion of nonsense- mediated mRNA decay. Cell. 2010;143:938-50 pubmed publisher
    ..Here, we demonstrate that the ATPase activity of the RNA helicase Upf1 allows disassembly of mRNPs undergoing nonsense-mediated mRNA decay (NMD)...
  33. Chen Y, Bennett C, Huynh H, Blair I, Puls I, Irobi J, et al. DNA/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4). Am J Hum Genet. 2004;74:1128-35 pubmed
    ..Although its function remains unknown, SETX contains a DNA/RNA helicase domain with strong homology to human RENT1 and IGHMBP2, two genes encoding proteins known to have roles in RNA processing...
  34. Kashima I, Jonas S, Jayachandran U, Buchwald G, Conti E, Lupas A, et al. SMG6 interacts with the exon junction complex via two conserved EJC-binding motifs (EBMs) required for nonsense-mediated mRNA decay. Genes Dev. 2010;24:2440-50 pubmed publisher
  35. Chamieh H, Ballut L, Bonneau F, Le Hir H. NMD factors UPF2 and UPF3 bridge UPF1 to the exon junction complex and stimulate its RNA helicase activity. Nat Struct Mol Biol. 2008;15:85-93 pubmed
    ..mRNAs containing a premature translation termination codon through the recruitment of the conserved NMD factors UPF1, UPF2 and UPF3...
  36. Lykke Andersen J. Identification of a human decapping complex associated with hUpf proteins in nonsense-mediated decay. Mol Cell Biol. 2002;22:8114-21 pubmed
    ..coimmunoprecipitation assays demonstrate that hDcp1a and hDcp2 interact with the nonsense-mediated decay factor hUpf1, both in the presence and in the absence of the other hUpf proteins, hUpf2, hUpf3a, and hUpf3b...
  37. Serin G, Gersappe A, Black J, Aronoff R, Maquat L. Identification and characterization of human orthologues to Saccharomyces cerevisiae Upf2 protein and Upf3 protein (Caenorhabditis elegans SMG-4). Mol Cell Biol. 2001;21:209-23 pubmed
    ..To date, human (h) Upf1 protein (p) (hUpf1p), a group 1 RNA helicase named after its Saccharomyces cerevisiae orthologue that functions in ..
  38. Perlick H, Medghalchi S, Spencer F, Kendzior R, Dietz H. Mammalian orthologues of a yeast regulator of nonsense transcript stability. Proc Natl Acad Sci U S A. 1996;93:10928-32 pubmed
    ..We have cloned cDNAs encoding human and murine homologues of Upf1p, termed rent1 (regulator of nonsense transcripts)...
  39. Arias Palomo E, Yamashita A, Fernández I, Núñez Ramírez R, Bamba Y, Izumi N, et al. The nonsense-mediated mRNA decay SMG-1 kinase is regulated by large-scale conformational changes controlled by SMG-8. Genes Dev. 2011;25:153-64 pubmed publisher
    ..SMG-1 is a large kinase essential to NMD that phosphorylates Upf1, which seems to be the definitive signal triggering mRNA decay...
  40. Flury V, Restuccia U, Bachi A, Mühlemann O. Characterization of phosphorylation- and RNA-dependent UPF1 interactors by quantitative proteomics. J Proteome Res. 2014;13:3038-53 pubmed publisher
    Human up-frameshift 1 (UPF1) is an ATP-dependent RNA helicase and phosphoprotein implicated in several biological processes but is best known for its key function in nonsense-mediated mRNA decay (NMD)...
  41. Shigeoka T, Kato S, Kawaichi M, Ishida Y. Evidence that the Upf1-related molecular motor scans the 3'-UTR to ensure mRNA integrity. Nucleic Acids Res. 2012;40:6887-97 pubmed publisher
    b>Upf1 is a highly conserved RNA helicase essential for nonsense-mediated mRNA decay (NMD), an mRNA quality-control mechanism that degrades aberrant mRNAs harboring premature termination codons (PTCs)...
  42. Agranat L, Raitskin O, Sperling J, Sperling R. The editing enzyme ADAR1 and the mRNA surveillance protein hUpf1 interact in the cell nucleus. Proc Natl Acad Sci U S A. 2008;105:5028-33 pubmed publisher
    ..we show that the A-to-I RNA editing protein adenosine deaminase that acts on RNA 1 (ADAR1) and the human Upf1 (hUpf1) protein involved in RNA surveillance are found associated within nuclear RNA-splicing complexes...
  43. Fanourgakis G, Lesche M, Akpinar M, Dahl A, Jessberger R. Chromatoid Body Protein TDRD6 Supports Long 3' UTR Triggered Nonsense Mediated mRNA Decay. PLoS Genet. 2016;12:e1005857 pubmed publisher
    ..Proteome analysis of purified CBs revealed components of the nonsense-mediated mRNA decay (NMD) machinery including UPF1. TDRD6 is essential for UPF1 localization to CBs, for UPF1-UPF2 and UPF1-MVH interactions...
  44. Hug N, Cáceres J. The RNA helicase DHX34 activates NMD by promoting a transition from the surveillance to the decay-inducing complex. Cell Rep. 2014;8:1845-1856 pubmed publisher
    Nonsense-mediated decay (NMD) is a surveillance mechanism that degrades aberrant mRNAs. A complex comprising SMG1, UPF1, and the translation termination factors eRF1 and eRF3 (SURF) is assembled in the vicinity of a premature termination ..
  45. Mendell J, Medghalchi S, Lake R, Noensie E, Dietz H. Novel Upf2p orthologues suggest a functional link between translation initiation and nonsense surveillance complexes. Mol Cell Biol. 2000;20:8944-57 pubmed
    ..rent2 was demonstrated to interact directly with rent1, a known trans-effector of NMD in mammalian cells...
  46. Schweingruber C, Rufener S, Zünd D, Yamashita A, Mühlemann O. Nonsense-mediated mRNA decay - mechanisms of substrate mRNA recognition and degradation in mammalian cells. Biochim Biophys Acta. 2013;1829:612-23 pubmed publisher
    ..In addition, the central role of UPF1, its crucial phosphorylation/dephosphorylation cycle and dynamic interactions with other NMD factors are discussed...
  47. Cho H, Park O, Park J, Ryu I, Kim J, Ko J, et al. Glucocorticoid receptor interacts with PNRC2 in a ligand-dependent manner to recruit UPF1 for rapid mRNA degradation. Proc Natl Acad Sci U S A. 2015;112:E1540-9 pubmed publisher
    ..Here, we demonstrate that GR, preloaded onto the 5'UTR of a target mRNA, recruits UPF1 through proline-rich nuclear receptor coregulatory protein 2 (PNRC2) in a ligand-dependent manner, so as to elicit ..
  48. Zupanic A, Méplan C, Huguenin G, Hesketh J, Shanley D. Modeling and gene knockdown to assess the contribution of nonsense-mediated decay, premature termination, and selenocysteine insertion to the selenoprotein hierarchy. RNA. 2016;22:1076-84 pubmed publisher
    ..The model predictions were tested using SiRNA to knock down expression of the crucial NMD factor UPF1 (up-frameshift protein 1) and selenoprotein mRNA expression...
  49. de Turris V, Nicholson P, Orozco R, Singer R, Mühlemann O. Cotranscriptional effect of a premature termination codon revealed by live-cell imaging. RNA. 2011;17:2094-107 pubmed publisher
    ..retained PTC+ transcripts are mainly unspliced, and this RNA retention is dependent upon two important NMD factors, UPF1 and SMG6, since their depletion led to the release of the PTC+ transcripts...
  50. Sugiyama T, Nobuta R, Ando K, Matsuki Y, Inada T. Crucial role of ATP-bound Sse1 in Upf1-dependent degradation of the truncated product. Biochem Biophys Res Commun. 2017;488:122-128 pubmed publisher
    ..We propose that the Sse1/Hsp70 complex maintains the solubility of FLAG-Pgk1-300, thereby stimulating its Upf-dependent degradation by the proteasomes. ..
  51. Hosoda N, Kim Y, Lejeune F, Maquat L. CBP80 promotes interaction of Upf1 with Upf2 during nonsense-mediated mRNA decay in mammalian cells. Nat Struct Mol Biol. 2005;12:893-901 pubmed
    ..An EJC includes the NMD factors Upf3 or Upf3X and Upf2, and Upf2 recruits Upf1. Once this pioneer translation initiation complex is remodeled so that CBP80-CBP20 is replaced by eukaryotic ..
  52. Melero R, Uchiyama A, Castaño R, Kataoka N, Kurosawa H, Ohno S, et al. Structures of SMG1-UPFs complexes: SMG1 contributes to regulate UPF2-dependent activation of UPF1 in NMD. Structure. 2014;22:1105-1119 pubmed publisher
    ..SMG1-mediated phosphorylation of the UPF1 helicase is an essential step during NMD initiation...
  53. Jin H, Suh M, Han J, Yeom K, Lee Y, Heo I, et al. Human UPF1 participates in small RNA-induced mRNA downregulation. Mol Cell Biol. 2009;29:5789-99 pubmed publisher
    ..Here we identify human UPF1 (hUPF1) as a protein that contributes to RNA silencing. When hUPF1 is knocked down, miRNA targets are upregulated...
  54. Ottens F, Boehm V, Sibley C, Ule J, Gehring N. Transcript-specific characteristics determine the contribution of endo- and exonucleolytic decay pathways during the degradation of nonsense-mediated decay substrates. RNA. 2017;23:1224-1236 pubmed publisher
    ..Degradation of NMD substrates is initiated by the central NMD factor UPF1, which recruits the endonuclease SMG6 and the deadenylation-promoting SMG5/7 complex...
  55. Brichta L, Garbes L, Jedrzejowska M, Grellscheid S, Holker I, Zimmermann K, et al. Nonsense-mediated messenger RNA decay of survival motor neuron 1 causes spinal muscular atrophy. Hum Genet. 2008;123:141-53 pubmed publisher
    ..A similar effect was observed after siRNA-mediated knockdown of UPF1, a factor essential for NMD...
  56. Fiorini F, Robin J, Kanaan J, Borowiak M, Croquette V, Le Hir H, et al. HTLV-1 Tax plugs and freezes UPF1 helicase leading to nonsense-mediated mRNA decay inhibition. Nat Commun. 2018;9:431 pubmed publisher
    Up-Frameshift Suppressor 1 Homolog (UPF1) is a key factor for nonsense-mediated mRNA decay (NMD), a cellular process that can actively degrade mRNAs...
  57. Kim W, Yun S, Kwon Y, You K, Shin N, Kim J, et al. mRNAs containing NMD-competent premature termination codons are stabilized and translated under UPF1 depletion. Sci Rep. 2017;7:15833 pubmed publisher
    ..The generation of mutant proteins was promoted by UPF1 depletion, which induced polysome association of PTC-containing mRNAs, increased eIF4E-bound PTC-containing mRNA ..
  58. He F, Jacobson A. Nonsense-Mediated mRNA Decay: Degradation of Defective Transcripts Is Only Part of the Story. Annu Rev Genet. 2015;49:339-66 pubmed publisher
    ..From yeasts to humans, activation of NMD requires the function of the three conserved Upf factors: Upf1, Upf2, and Upf3...
  59. Hoque M, Park J, Chang Y, Luchessi A, Cambiaghi T, Shamanna R, et al. Regulation of gene expression by translation factor eIF5A: Hypusine-modified eIF5A enhances nonsense-mediated mRNA decay in human cells. Translation (Austin). 2017;5:e1366294 pubmed publisher
    ..genes whose expression is coordinately regulated by eIF5A1, its modifying enzymes, and the pivotal NMD factor, Upf1. Transcripts encoding components of the translation system were highly represented, including some encoding ..
  60. Feng Q, Jagannathan S, Bradley R. The RNA Surveillance Factor UPF1 Represses Myogenesis via Its E3 Ubiquitin Ligase Activity. Mol Cell. 2017;67:239-251.e6 pubmed publisher
    b>UPF1 is an RNA helicase that orchestrates nonsense-mediated decay and other RNA surveillance pathways...
  61. Liu C, Karam R, Zhou Y, Su F, Ji Y, Li G, et al. The UPF1 RNA surveillance gene is commonly mutated in pancreatic adenosquamous carcinoma. Nat Med. 2014;20:596-8 pubmed publisher
    ..Here we report that ASC tumors frequently harbor somatically acquired mutations in the UPF1 gene, which encodes the core component of the nonsense-mediated RNA decay (NMD) pathway...
  62. Colombo M, Karousis E, Bourquin J, Bruggmann R, Mühlemann O. Transcriptome-wide identification of NMD-targeted human mRNAs reveals extensive redundancy between SMG6- and SMG7-mediated degradation pathways. RNA. 2017;23:189-201 pubmed publisher
    ..approach in which we combined transcriptome profiling of knockdowns and rescues of the three NMD factors UPF1, SMG6, and SMG7...
  63. Carastro L, Tan C, Selg M, Jack H, So A, Downey K. Identification of delta helicase as the bovine homolog of HUPF1: demonstration of an interaction with the third subunit of DNA polymerase delta. Nucleic Acids Res. 2002;30:2232-43 pubmed
    b>Delta helicase is a 5' to 3' DNA helicase that partially co-purifies with DNA polymerase delta (pol delta) from fetal bovine thymus tissue...
  64. Dankert J, Róna G, Clijsters L, Geter P, Skaar J, Bermudez Hernandez K, et al. Cyclin F-Mediated Degradation of SLBP Limits H2A.X Accumulation and Apoptosis upon Genotoxic Stress in G2. Mol Cell. 2016;64:507-519 pubmed publisher
    ..We propose that cyclin F co-evolved with the appearance of stem-loops in vertebrate H2AFX mRNA to mediate SLBP degradation, thereby limiting H2A.X synthesis and cell death upon genotoxic stress. ..
  65. Miki T, Kamikawa Y, Kurono S, Kaneko Y, Katahira J, Yoneda Y. Cell type-dependent gene regulation by Staufen2 in conjunction with Upf1. BMC Mol Biol. 2011;12:48 pubmed publisher
    ..To elucidate the functions of Stau2, we screened for novel binding partners by affinity purification of GST-tagged Stau2 from 293F cells...
  66. Barmada S, Ju S, Arjun A, Batarse A, Archbold H, Peisach D, et al. Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1. Proc Natl Acad Sci U S A. 2015;112:7821-6 pubmed publisher
    ..primary neuronal model of ALS and FTD to investigate and characterize the role of human up-frameshift protein 1 (hUPF1), an RNA helicase and master regulator of NMD, in these disorders...
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