Published in Mol Cell on October 07, 2005
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) 5.59
NMD: a multifaceted response to premature translational termination. Nat Rev Mol Cell Biol (2012) 3.64
Messenger RNA regulation: to translate or to degrade. EMBO J (2008) 3.38
The multiple lives of NMD factors: balancing roles in gene and genome regulation. Nat Rev Genet (2008) 3.00
A competition between stimulators and antagonists of Upf complex recruitment governs human nonsense-mediated mRNA decay. PLoS Biol (2008) 2.61
Organizing principles of mammalian nonsense-mediated mRNA decay. Annu Rev Genet (2013) 2.55
Upf1 ATPase-dependent mRNP disassembly is required for completion of nonsense- mediated mRNA decay. Cell (2010) 2.53
Interactions between UPF1, eRFs, PABP and the exon junction complex suggest an integrated model for mammalian NMD pathways. EMBO J (2008) 2.52
Mutations in UPF3B, a member of the nonsense-mediated mRNA decay complex, cause syndromic and nonsyndromic mental retardation. Nat Genet (2007) 2.35
Nonsense-mediated mRNA decay (NMD) mechanisms. Nat Struct Mol Biol (2009) 2.31
NMD is essential for hematopoietic stem and progenitor cells and for eliminating by-products of programmed DNA rearrangements. Genes Dev (2008) 2.26
Regulation of multiple core spliceosomal proteins by alternative splicing-coupled nonsense-mediated mRNA decay. Mol Cell Biol (2008) 2.25
Structures of the PIN domains of SMG6 and SMG5 reveal a nuclease within the mRNA surveillance complex. EMBO J (2006) 2.16
A conserved role for cytoplasmic poly(A)-binding protein 1 (PABPC1) in nonsense-mediated mRNA decay. EMBO J (2007) 2.11
UPF1 association with the cap-binding protein, CBP80, promotes nonsense-mediated mRNA decay at two distinct steps. Mol Cell (2010) 2.04
An alternative branch of the nonsense-mediated decay pathway. EMBO J (2007) 2.01
SMD and NMD are competitive pathways that contribute to myogenesis: effects on PAX3 and myogenin mRNAs. Genes Dev (2008) 1.97
Protein composition of human mRNPs spliced in vitro and differential requirements for mRNP protein recruitment. RNA (2006) 1.83
Nonsense-mediated mRNA decay affects nonsense transcript levels and governs response of cystic fibrosis patients to gentamicin. J Clin Invest (2007) 1.73
Nonsense-mediated mRNA decay: an intricate machinery that shapes transcriptomes. Nat Rev Mol Cell Biol (2015) 1.61
Regulation of nonsense-mediated mRNA decay: implications for physiology and disease. Biochim Biophys Acta (2013) 1.59
The abundance of RNPS1, a protein component of the exon junction complex, can determine the variability in efficiency of the Nonsense Mediated Decay pathway. Nucleic Acids Res (2007) 1.53
Nonsense-mediated mRNA decay (NMD) in animal embryogenesis: to die or not to die, that is the question. Curr Opin Genet Dev (2011) 1.52
RNA homeostasis governed by cell type-specific and branched feedback loops acting on NMD. Mol Cell (2011) 1.50
Inhibition of nonsense-mediated mRNA decay (NMD) by a new chemical molecule reveals the dynamic of NMD factors in P-bodies. J Cell Biol (2007) 1.47
Functions of hUpf3a and hUpf3b in nonsense-mediated mRNA decay and translation. RNA (2006) 1.46
Nonsense-mediated mRNA decay effectors are essential for zebrafish embryonic development and survival. Mol Cell Biol (2009) 1.44
Unusual bipartite mode of interaction between the nonsense-mediated decay factors, UPF1 and UPF2. EMBO J (2009) 1.44
Auto- and cross-regulation of the hnRNP L proteins by alternative splicing. Mol Cell Biol (2009) 1.41
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Antagonistic factors control the unproductive splicing of SC35 terminal intron. Nucleic Acids Res (2009) 1.28
The hierarchy of exon-junction complex assembly by the spliceosome explains key features of mammalian nonsense-mediated mRNA decay. PLoS Biol (2009) 1.24
Therapeutics based on stop codon readthrough. Annu Rev Genomics Hum Genet (2014) 1.23
HPat provides a link between deadenylation and decapping in metazoa. J Cell Biol (2010) 1.23
Caenorhabditis elegans SMG-2 selectively marks mRNAs containing premature translation termination codons. Mol Cell Biol (2007) 1.18
A UPF3-mediated regulatory switch that maintains RNA surveillance. Nat Struct Mol Biol (2009) 1.16
CUP promotes deadenylation and inhibits decapping of mRNA targets. Genes Dev (2011) 1.14
Insights into the recruitment of the NMD machinery from the crystal structure of a core EJC-UPF3b complex. Proc Natl Acad Sci U S A (2010) 1.14
Posttranscriptional control of the stem cell and neurogenic programs by the nonsense-mediated RNA decay pathway. Cell Rep (2014) 1.13
Mutational analysis of human eIF4AIII identifies regions necessary for exon junction complex formation and nonsense-mediated mRNA decay. RNA (2006) 1.13
Rescue of nonsense mutations by amlexanox in human cells. Orphanet J Rare Dis (2012) 1.13
Expression proteomics of UPF1 knockdown in HeLa cells reveals autoregulation of hnRNP A2/B1 mediated by alternative splicing resulting in nonsense-mediated mRNA decay. BMC Genomics (2010) 1.07
Comparison of EJC-enhanced and EJC-independent NMD in human cells reveals two partially redundant degradation pathways. RNA (2013) 1.07
Splicing-dependent NMD does not require the EJC in Schizosaccharomyces pombe. EMBO J (2010) 1.06
Upf1 potentially serves as a RING-related E3 ubiquitin ligase via its association with Upf3 in yeast. RNA (2008) 1.06
Aberrant splicing of the E-cadherin transcript is a novel mechanism of gene silencing in chronic lymphocytic leukemia cells. Blood (2009) 1.05
The unfolded protein response is shaped by the NMD pathway. EMBO Rep (2015) 1.05
The cryo-EM structure of the UPF-EJC complex shows UPF1 poised toward the RNA 3' end. Nat Struct Mol Biol (2012) 1.04
The uORF-containing thrombopoietin mRNA escapes nonsense-mediated decay (NMD). Nucleic Acids Res (2006) 1.03
Turnover in the Alps: an mRNA perspective. Workshops on mechanisms and regulation of mRNA turnover. EMBO Rep (2006) 1.03
Nonsense-mediated mRNA decay in humans at a glance. J Cell Sci (2016) 1.01
mRNA-mRNA duplexes that autoelicit Staufen1-mediated mRNA decay. Nat Struct Mol Biol (2013) 1.01
Mechanism and regulation of the nonsense-mediated decay pathway. Nucleic Acids Res (2016) 0.99
The exon junction complex as a node of post-transcriptional networks. Nat Rev Mol Cell Biol (2015) 0.99
Transcriptome profiling of UPF3B/NMD-deficient lymphoblastoid cells from patients with various forms of intellectual disability. Mol Psychiatry (2011) 0.99
SMG5-PNRC2 is functionally dominant compared with SMG5-SMG7 in mammalian nonsense-mediated mRNA decay. Nucleic Acids Res (2012) 0.98
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Gene expression networks: competing mRNA decay pathways in mammalian cells. Biochem Soc Trans (2009) 0.96
Inhibition of nonsense-mediated mRNA decay by the natural product pateamine A through eukaryotic initiation factor 4AIII. J Biol Chem (2009) 0.96
Regulation of H-ras splice variant expression by cross talk between the p53 and nonsense-mediated mRNA decay pathways. Mol Cell Biol (2007) 0.96
Drosophila Upf1 and Upf2 loss of function inhibits cell growth and causes animal death in a Upf3-independent manner. RNA (2011) 0.96
Biochemical and cellular characterization of the plant ortholog of PYM, a protein that interacts with the exon junction complex core proteins Mago and Y14. Planta (2006) 0.96
Nonsense codons trigger an RNA partitioning shift. J Biol Chem (2008) 0.96
Chapter 3. Assays of adenylate uridylate-rich element-mediated mRNA decay in cells. Methods Enzymol (2008) 0.95
Perispeckles are major assembly sites for the exon junction core complex. Mol Biol Cell (2012) 0.95
The structure of the ASAP core complex reveals the existence of a Pinin-containing PSAP complex. Nat Struct Mol Biol (2012) 0.95
Nonsense-mediated mRNA decay: inter-individual variability and human disease. Neurosci Biobehav Rev (2013) 0.94
The interaction of cytoplasmic poly(A)-binding protein with eukaryotic initiation factor 4G suppresses nonsense-mediated mRNA decay. RNA (2014) 0.94
Upf1/Upf2 regulation of 3' untranslated region splice variants of AUF1 links nonsense-mediated and A+U-rich element-mediated mRNA decay. Mol Cell Biol (2006) 0.93
Two mammalian MAGOH genes contribute to exon junction complex composition and nonsense-mediated decay. RNA Biol (2013) 0.92
5-azacytidine inhibits nonsense-mediated decay in a MYC-dependent fashion. EMBO Mol Med (2014) 0.91
The spliceosome-activating complex: molecular mechanisms underlying the function of a pleiotropic regulator. Front Plant Sci (2012) 0.90
Nonsense-mediated mRNA decay impacts MSI-driven carcinogenesis and anti-tumor immunity in colorectal cancers. PLoS One (2008) 0.90
Method for quantitative analysis of nonsense-mediated mRNA decay at the single cell level. Sci Rep (2015) 0.90
DHX34 and NBAS form part of an autoregulatory NMD circuit that regulates endogenous RNA targets in human cells, zebrafish and Caenorhabditis elegans. Nucleic Acids Res (2013) 0.89
Identification of elements in human long 3' UTRs that inhibit nonsense-mediated decay. RNA (2015) 0.88
Coupling pre-mRNA processing to transcription on the RNA factory assembly line. RNA Biol (2013) 0.87
Stress-sensitive regulation of IFRD1 mRNA decay is mediated by an upstream open reading frame. J Biol Chem (2010) 0.86
Identification and functional analysis of novel phosphorylation sites in the RNA surveillance protein Upf1. Nucleic Acids Res (2013) 0.86
The Antagonistic Gene Paralogs Upf3a and Upf3b Govern Nonsense-Mediated RNA Decay. Cell (2016) 0.86
Correction of mutations within the cystic fibrosis transmembrane conductance regulator by site-directed RNA editing. Proc Natl Acad Sci U S A (2013) 0.85
The RNA helicase DHX34 activates NMD by promoting a transition from the surveillance to the decay-inducing complex. Cell Rep (2014) 0.85
Identification of SMG6 cleavage sites and a preferred RNA cleavage motif by global analysis of endogenous NMD targets in human cells. Nucleic Acids Res (2014) 0.85
A conserved microRNA/NMD regulatory circuit controls gene expression. RNA Biol (2012) 0.84
Specific Y14 domains mediate its nucleo-cytoplasmic shuttling and association with spliced mRNA. Sci Rep (2011) 0.83
Structural and functional analysis of the three MIF4G domains of nonsense-mediated decay factor UPF2. Nucleic Acids Res (2013) 0.83
Caspases shutdown nonsense-mediated mRNA decay during apoptosis. Cell Death Differ (2015) 0.82
A critical role of RBM8a in proliferation and differentiation of embryonic neural progenitors. Neural Dev (2015) 0.82
Integrin α3β1 controls mRNA splicing that determines Cox-2 mRNA stability in breast cancer cells. J Cell Sci (2014) 0.81
Post-transcriptional regulation of 5-lipoxygenase mRNA expression via alternative splicing and nonsense-mediated mRNA decay. PLoS One (2012) 0.81
UPF1 is crucial for the infectivity of human immunodeficiency virus type 1 progeny virions. J Virol (2013) 0.81
Dual function of UPF3B in early and late translation termination. EMBO J (2017) 0.81
The Supraspliceosome - A Multi-Task Machine for Regulated Pre-mRNA Processing in the Cell Nucleus. Comput Struct Biotechnol J (2014) 0.80
Exon Junction Complexes Show a Distributional Bias toward Alternatively Spliced mRNAs and against mRNAs Coding for Ribosomal Proteins. Cell Rep (2016) 0.79
Identification and characterization of novel factors that act in the nonsense-mediated mRNA decay pathway in nematodes, flies and mammals. EMBO Rep (2014) 0.79
Function and pathological implications of exon junction complex factor Y14. Biomolecules (2015) 0.79
The RNA helicase Ddx5/p68 binds to hUpf3 and enhances NMD of Ddx17/p72 and Smg5 mRNA. Nucleic Acids Res (2013) 0.79
The Exon Junction Complex Controls the Efficient and Faithful Splicing of a Subset of Transcripts Involved in Mitotic Cell-Cycle Progression. Int J Mol Sci (2016) 0.78
Driver mutations in histone H3.3 and chromatin remodelling genes in paediatric glioblastoma. Nature (2012) 10.99
Hotspot mutations in H3F3A and IDH1 define distinct epigenetic and biological subgroups of glioblastoma. Cancer Cell (2012) 6.71
Molecular mechanisms of translational control. Nat Rev Mol Cell Biol (2004) 6.39
Insights into RNA biology from an atlas of mammalian mRNA-binding proteins. Cell (2012) 6.22
Genome sequencing of pediatric medulloblastoma links catastrophic DNA rearrangements with TP53 mutations. Cell (2012) 6.07
Nonsense-mediated decay approaches the clinic. Nat Genet (2004) 5.12
Dissecting the genomic complexity underlying medulloblastoma. Nature (2012) 4.77
Drosophila miR2 induces pseudo-polysomes and inhibits translation initiation. Nature (2007) 3.77
A sensitive array for microRNA expression profiling (miChip) based on locked nucleic acids (LNA). RNA (2006) 3.71
A conserved motif in Argonaute-interacting proteins mediates functional interactions through the Argonaute PIWI domain. Nat Struct Mol Biol (2007) 3.62
Phosphorylation of hUPF1 induces formation of mRNA surveillance complexes containing hSMG-5 and hSMG-7. Mol Cell (2003) 3.53
STAT3 mediates hepatic hepcidin expression and its inflammatory stimulation. Blood (2006) 3.38
FSTL5 is a marker of poor prognosis in non-WNT/non-SHH medulloblastoma. J Clin Oncol (2011) 3.33
Y14 and hUpf3b form an NMD-activating complex. Mol Cell (2003) 3.17
Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma. Cancer Cell (2011) 3.11
Systemic iron homeostasis and the iron-responsive element/iron-regulatory protein (IRE/IRP) regulatory network. Annu Rev Nutr (2008) 2.98
Bruno acts as a dual repressor of oskar translation, promoting mRNA oligomerization and formation of silencing particles. Cell (2006) 2.77
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Gain-of-function mutations in interleukin-7 receptor-α (IL7R) in childhood acute lymphoblastic leukemias. J Exp Med (2011) 2.59
Interactions between UPF1, eRFs, PABP and the exon junction complex suggest an integrated model for mammalian NMD pathways. EMBO J (2008) 2.52
Regulatory defects in liver and intestine implicate abnormal hepcidin and Cybrd1 expression in mouse hemochromatosis. Nat Genet (2003) 2.43
Recurrent somatic alterations of FGFR1 and NTRK2 in pilocytic astrocytoma. Nat Genet (2013) 2.42
The liver-specific microRNA miR-122 controls systemic iron homeostasis in mice. J Clin Invest (2011) 2.30
Molecular staging of intracranial ependymoma in children and adults. J Clin Oncol (2010) 2.27
Bone morphogenetic protein (BMP)-responsive elements located in the proximal and distal hepcidin promoter are critical for its response to HJV/BMP/SMAD. J Mol Med (Berl) (2009) 2.15
The role of ABCE1 in eukaryotic posttermination ribosomal recycling. Mol Cell (2010) 2.14
The RNA-binding protein repertoire of embryonic stem cells. Nat Struct Mol Biol (2013) 2.12
Activating NOTCH1 mutations predict favorable early treatment response and long-term outcome in childhood precursor T-cell lymphoblastic leukemia. Blood (2006) 2.10
NMD: RNA biology meets human genetic medicine. Biochem J (2010) 2.09
Adult medulloblastoma comprises three major molecular variants. J Clin Oncol (2011) 2.07
Serum ferritin is derived primarily from macrophages through a nonclassical secretory pathway. Blood (2010) 2.02
c-Src-mediated phosphorylation of hnRNP K drives translational activation of specifically silenced mRNAs. Mol Cell Biol (2002) 1.98
Iron-regulatory proteins limit hypoxia-inducible factor-2alpha expression in iron deficiency. Nat Struct Mol Biol (2007) 1.96
Homodirectional changes in transcriptome composition and mRNA translation induced by rapamycin and heat shock. Nat Struct Biol (2003) 1.95
PABP and the poly(A) tail augment microRNA repression by facilitated miRISC binding. Nat Struct Mol Biol (2012) 1.91
A dual inhibitory mechanism restricts msl-2 mRNA translation for dosage compensation in Drosophila. Cell (2005) 1.91
Oncogenic FAM131B-BRAF fusion resulting from 7q34 deletion comprises an alternative mechanism of MAPK pathway activation in pilocytic astrocytoma. Acta Neuropathol (2011) 1.90
Cartilage-like gene expression in differentiated human stem cell spheroids: a comparison of bone marrow-derived and adipose tissue-derived stromal cells. Arthritis Rheum (2003) 1.84
TP53 mutation is frequently associated with CTNNB1 mutation or MYCN amplification and is compatible with long-term survival in medulloblastoma. J Clin Oncol (2010) 1.83
Focal genomic amplification at 19q13.42 comprises a powerful diagnostic marker for embryonal tumors with ependymoblastic rosettes. Acta Neuropathol (2010) 1.82
Drosophila miR2 primarily targets the m7GpppN cap structure for translational repression. Mol Cell (2009) 1.82
Previously uncharacterized isoforms of divalent metal transporter (DMT)-1: implications for regulation and cellular function. Proc Natl Acad Sci U S A (2002) 1.77
Translational control via protein-regulated upstream open reading frames. Cell (2011) 1.76
Complexes between the nonsense-mediated mRNA decay pathway factor human upf1 (up-frameshift protein 1) and essential nonsense-mediated mRNA decay factors in HeLa cells. Biochem J (2003) 1.74
Nonsense-mediated mRNA decay affects nonsense transcript levels and governs response of cystic fibrosis patients to gentamicin. J Clin Invest (2007) 1.73
Ca2+ channel blockers reverse iron overload by a new mechanism via divalent metal transporter-1. Nat Med (2007) 1.73
A bone morphogenetic protein (BMP)-responsive element in the hepcidin promoter controls HFE2-mediated hepatic hepcidin expression and its response to IL-6 in cultured cells. J Mol Med (Berl) (2008) 1.71
Disassembly of exon junction complexes by PYM. Cell (2009) 1.69
An Hfe-dependent pathway mediates hyposideremia in response to lipopolysaccharide-induced inflammation in mice. Nat Genet (2004) 1.68
microRNA-mediated messenger RNA deadenylation contributes to translational repression in mammalian cells. PLoS One (2009) 1.67
Adult and pediatric medulloblastomas are genetically distinct and require different algorithms for molecular risk stratification. J Clin Oncol (2010) 1.65
Altered body iron distribution and microcytosis in mice deficient in iron regulatory protein 2 (IRP2). Blood (2005) 1.59
Nonsense-mediated mRNA decay: from vacuum cleaner to Swiss army knife. Genome Biol (2004) 1.59
Integration of splicing, transport and translation to achieve mRNA quality control by the nonsense-mediated decay pathway. Genome Biol (2002) 1.58
Hfe acts in hepatocytes to prevent hemochromatosis. Cell Metab (2008) 1.55
Iron regulatory proteins are essential for intestinal function and control key iron absorption molecules in the duodenum. Cell Metab (2008) 1.54
The abundance of RNPS1, a protein component of the exon junction complex, can determine the variability in efficiency of the Nonsense Mediated Decay pathway. Nucleic Acids Res (2007) 1.53
Using the lambdaN peptide to tether proteins to RNAs. Methods Mol Biol (2004) 1.52
Unbiased RNAi screen for hepcidin regulators links hepcidin suppression to proliferative Ras/RAF and nutrient-dependent mTOR signaling. Blood (2014) 1.50
The efficiency of nonsense-mediated mRNA decay is an inherent character and varies among different cells. Eur J Hum Genet (2007) 1.48
Mechanism of translational regulation by miR-2 from sites in the 5' untranslated region or the open reading frame. RNA (2010) 1.46
Functions of hUpf3a and hUpf3b in nonsense-mediated mRNA decay and translation. RNA (2006) 1.46
Unusual bipartite mode of interaction between the nonsense-mediated decay factors, UPF1 and UPF2. EMBO J (2009) 1.44
Abnormally spliced beta-globin mRNAs: a single point mutation generates transcripts sensitive and insensitive to nonsense-mediated mRNA decay. Blood (2002) 1.43
miChip: an array-based method for microRNA expression profiling using locked nucleic acid capture probes. Nat Protoc (2008) 1.43
RNA-binding proteins in Mendelian disease. Trends Genet (2013) 1.38
Iron-mediated degradation of IRP2, an unexpected pathway involving a 2-oxoglutarate-dependent oxygenase activity. Mol Cell Biol (2004) 1.37
Eukaryotic translation initiation factor 4GI and p97 promote cellular internal ribosome entry sequence-driven translation. Proc Natl Acad Sci U S A (2005) 1.36
Enhancement of IRES-mediated translation of the c-myc and BiP mRNAs by the poly(A) tail is independent of intact eIF4G and PABP. Mol Cell (2004) 1.36
Integrated analysis of pediatric glioblastoma reveals a subset of biologically favorable tumors with associated molecular prognostic markers. Acta Neuropathol (2015) 1.35
SMAD7 controls iron metabolism as a potent inhibitor of hepcidin expression. Blood (2009) 1.33
Biological and clinical heterogeneity of MYCN-amplified medulloblastoma. Acta Neuropathol (2011) 1.32
The hemochromatosis proteins HFE, TfR2, and HJV form a membrane-associated protein complex for hepcidin regulation. J Hepatol (2012) 1.28
Splicing factors stimulate polyadenylation via USEs at non-canonical 3' end formation signals. EMBO J (2007) 1.27
Enhanced expression of the human chitinase 3-like 2 gene (YKL-39) but not chitinase 3-like 1 gene (YKL-40) in osteoarthritic cartilage. Biochem Biophys Res Commun (2002) 1.27
Iron regulation and the cell cycle: identification of an iron-responsive element in the 3'-untranslated region of human cell division cycle 14A mRNA by a refined microarray-based screening strategy. J Biol Chem (2006) 1.25