When cells stop making sense: effects of nonsense codons on RNA metabolism in vertebrate cells.

A novel form of tissue-specific RNA processing produces apolipoprotein-B48 in intestine

1987 • 1,021 citations

Apolipoprotein B-48 Is the Product of a Messenger RNA with an Organ-Specific In-Frame Stop Codon

1987 • 746 citations

[16] Splice junctions, branch point sites, and exons: Sequence statistics, identification, and applications to genome project

1990 • 689 citations

A turnover pathway for both stable and unstable mRNAs in yeast: evidence for a requirement for deadenylation.

1993 • 660 citations

Degradation of mRNA in eukaryotes

1995 • 648 citations

Gene deletions causing human genetic disease: mechanisms of mutagenesis and the role of the local DNA sequence environment

1991 • 491 citations

The product of the yeast UPF1 gene is required for rapid turnover of mRNAs containing a premature translational termination codon.

1991 • 487 citations

mRNA surveillance by the Caenorhabditis elegans smg genes.

1993 • 459 citations

The Skipping of Constitutive Exons in Vivo Induced by Nonsense Mutations

1993 • 440 citations

Some cis- and trans-acting mutants for splicing target pre-mRNA to the cytoplasm

1989 • 417 citations

THE MUTATION AND POLYMORPHISM OF THE HUMAN β-GLOBIN GENE AND ITS SURROUNDING DNA

1984 • 404 citations

Premature translational termination triggers mRNA decapping

1994 • 399 citations

Yeast cells lacking 5'-->3' exoribonuclease 1 contain mRNA species that are poly(A) deficient and partially lack the 5' cap structure.

1993 • 378 citations

Nonsense mutations in the dihydrofolate reductase gene affect RNA processing.

1989 • 367 citations

Turnover Mechanisms of the Stable Yeast PGK1 mRNA

1995 • 341 citations

Gene products that promote mRNA turnover in Saccharomyces cerevisiae.

1992 • 330 citations

Regulation of Selenoproteins

1993 • 314 citations

mRNA destabilization triggered by premature translational termination depends on at least three cis-acting sequence elements and one trans-acting factor.

1993 • 307 citations

beta 0 thalassemia, a nonsense mutation in man.

1979 • 291 citations

Identification and characterization of genes that are required for the accelerated degradation of mRNAs containing a premature translational termination codon.

1995 • 279 citations

Identification of a novel component of the nonsense-mediated mRNA decay pathway by use of an interacting protein screen.

1995 • 278 citations

A new kind of informational suppression in the nematode Caenorhabditis elegans.

1989 • 272 citations

Unstable β-globin mRNA in mRNA-deficient β0 thalassemia

1981 • 267 citations

Stabilization and ribosome association of unspliced pre-mRNAs in a yeast upf1- mutant.

1993 • 265 citations

Translocation of a specific premessenger ribonucleoprotein particle through the nuclear pore studied with electron microscope tomography

1992 • 254 citations

Physical isolation of nascent RNA chains transcribed by RNA polymerase II: evidence for cotranscriptional splicing.

1994 • 230 citations

Premature translation termination mediates triosephosphate isomerase mRNA degradation.

1988 • 229 citations

Ribosome gymnastics—Degree of difficulty 9.5, style 10.0

1990 • 226 citations

The major defect in Ashkenazi Jews with Tay-Sachs disease is an insertion in the gene for the alpha-chain of beta-hexosaminidase.

1988 • 226 citations

Nonsense codons can reduce the abundance of nuclear mRNA without affecting the abundance of pre-mRNA or the half-life of cytoplasmic mRNA.

1993 • 200 citations

Consequences of frameshift mutations at the immunoglobulin heavy chain locus of the mouse.

1985 • 193 citations

Yeast Cells Lacking 5'→3' Exoribonuclease 1 Contain mRNA Species That are Poly(A) Deficient and Partially Lack the 5' Cap Structure

1993 • 190 citations

Conserved nucleotide sequences in the open reading frame and 3' untranslated region of selenoprotein P mRNA.

1993 • 182 citations

Evidence to implicate translation by ribosomes in the mechanism by which nonsense codons reduce the nuclear level of human triosephosphate isomerase mRNA.

1993 • 179 citations

Nonsense codon mutations in the terminal exon of the beta-globin gene are not associated with a reduction in beta-mRNA accumulation: a mechanism for the phenotype of dominant beta-thalassemia

1994 • 164 citations

Effect of dystrophin gene deletions on mRNA levels and processing in Duchenne and Becker muscular dystrophies

1990 • 160 citations

Translation to near the distal end of the penultimate exon is required for normal levels of spliced triosephosphate isomerase mRNA.

1990 • 159 citations

A nonsense mutation causing decreased levels of insulin receptor mRNA: detection by a simplified technique for direct sequencing of genomic DNA amplified by the polymerase chain reaction.

1990 • 155 citations

A single base deletion in the Tfm androgen receptor gene creates a short-lived messenger RNA that directs internal translation initiation.

1991 • 151 citations

Human alpha-L-iduronidase: cDNA isolation and expression.

1991 • 148 citations

Requirement for intron-encoded U22 small nucleolar RNA in 18S ribosomal RNA maturation

1994 • 146 citations

Maintenance of an open reading frame as an additional level of scrutiny during splice site selection

1994 • 146 citations

Characterization of cis-Acting Sequences and Decay Intermediates Involved in Nonsense-Mediated mRNA Turnover

1995 • 146 citations

Are vertebrate exons scanned during splice-site selection?

1992 • 141 citations

Mammalian nonsense codons can be cis effectors of nuclear mRNA half-life.

1994 • 140 citations

Nonsense Mutations in the Dihydrofolate Reductase Gene Affect RNA Processing

1989 • 139 citations

Identification and Characterization of a Sequence Motif Involved in Nonsense-Mediated mRNA Decay

1995 • 135 citations

Nonsense mutations inhibit splicing of MVM RNA in cis when they interrupt the reading frame of either exon of the final spliced product.

1992 • 135 citations

Intrinsic U2AF binding is modulated by exon enhancer signals in parallel with changes in splicing activity.

1995 • 133 citations

Gene Products That Promote mRNA Turnover in Saccharomyces cerevisiae

1992 • 132 citations

The majority of yeast UPF1 co-localizes with polyribosomes in the cytoplasm.

1995 • 132 citations

Introns are cis Effectors of the Nonsense-Codon-Mediated Reduction in Nuclear mRNA Abundance

1994 • 131 citations

Nonsense but not missense mutations can decrease the abundance of nuclear mRNA for the mouse major urinary protein, while both types of mutations can facilitate exon skipping.

1994 • 131 citations

Effect of premature termination of translation on mRNA stability depends on the site of ribosome release.

1987 • 127 citations

Beta-globin nonsense mutation: deficient accumulation of mRNA occurs despite normal cytoplasmic stability.

1992 • 124 citations

Diverse mutations in patients with Menkes disease often lead to exon skipping.

1994 • 123 citations

Analyis of factor VIII mRNA reveals defects in everyone of 28 haemophilia A patients

1993 • 123 citations

Nonsense Codons in Human β-Globin mRNA Result in the Production of mRNA Degradation Products

1992 • 118 citations

Translational Suppression in Retroviral GENE Expression

1992 • 118 citations

Physical Isolation of Nascent RNA Chains Transcribed by RNA Polymerase II: Evidence for Cotranscriptional Splicing

1994 • 114 citations

Nonsense-mediated mRNA Decay in Yeast

1994 • 109 citations

Structure and sequence of the human α-l-iduronidase gene

1992 • 109 citations

Beta O-39 thalassemia gene: a premature termination codon causes beta- mRNA deficiency without affecting cytoplasmic beta-mRNA stability

1984 • 106 citations

Intranuclear defect in beta-globin mRNA accumulation due to a premature translation termination codon

1984 • 102 citations

Upstream introns influence the efficiency of final intron removal and RNA 3'-end formation.

1994 • 101 citations

Low cytoplasmic mRNA levels of immunoglobulin kappa light chain genes containing nonsense codons correlate with inefficient splicing.

1994 • 97 citations

Order of intron removal during splicing of endogenous adenine phosphoribosyltransferase and dihydrofolate reductase pre-mRNA.

1993 • 97 citations

FRAMESHIFT SUPPRESSION IN SACCHAROMYCES CEREVISIAE. II. GENETIC PROPERTIES OF GROUP II SUPPRESSORS

1980 • 97 citations

DOMINANT β THALASSAEMIA: MOLECULAR BASIS AND PATHOPHYSIOLOGY

1992 • 95 citations

CFTR nonsense mutations G542X and W1282X associated with severe reduction of CFTR mRNA in nasal epithelial cells

1992 • 95 citations

Exon skipping and translation in patients with frameshift deletions in the dystrophin gene.

1993 • 93 citations

A nonsense mutation and exon skipping in the Fanconi anaemia group C gene

1993 • 91 citations

Human beta-globin mRNAs that harbor a nonsense codon are degraded in murine erythroid tissues to intermediates lacking regions of exon I or exons I and II that have a cap-like structure at the 5′ termini.

1992 • 90 citations

Molecular analysis of Hurler syndrome in Druze and Muslim Arab patients in Israel: multiple allelic mutations of the IDUA gene in a small geographic area.

1993 • 89 citations

Structure and expression of a cloned β°thalassaemic globin gene

1981 • 85 citations

Nonsense Codons Can Reduce the Abundance of Nuclear mRNA without Affecting the Abundance of Pre-mRNA or the Half-Life of Cytoplasmic mRNA

1993 • 82 citations

Premature Translation Termination Mediates Triosephosphate Isomerase mRNA Degradation

1988 • 82 citations

In vivo recognition of a vertebrate mini-exon as an exon-intron-exon unit.

1993 • 80 citations

Splicing Mutants and Their Second-Site Suppressors at the Dihydrofolate Reductase Locus in Chinese Hamster Ovary Cells

1993 • 77 citations

Emphysema associated with complete absence of alpha 1- antitrypsin in serum and the homozygous inheritance [corrected] of a stop codon in an alpha 1-antitrypsin-coding exon.

1988 • 77 citations

Nuclear degradation of nonsense mutated β-globin mRNA: a post-transcriptional mechanism to protect heterozygotes from severe clinical manifestations of β-thalassemia?

1995 • 73 citations

Dominant thalassemia-like phenotypes associated with mutations in exon 3 of the beta-globin gene.

1992 • 72 citations

Nonsense codons within the Rous sarcoma virus gag gene decrease the stability of unspliced viral RNA.

1991 • 72 citations

Human beta and delta globin messenger RNAs turn over at different rates

1983 • 72 citations

Origin of eukaryotic introns: a hypothesis, based on codon distribution statistics in genes, and its implications.

1986 • 70 citations

Determination of mRNA fate by different RNA polymerase II promoters.

1993 • 66 citations

Novel metabolism of several beta zero-thalassemic beta-globin mRNAs in the erythroid tissues of transgenic mice.

1989 • 56 citations

Translation to Near the Distal End of the Penultimate Exon Is Required for Normal Levels of Spliced Triosephosphate Isomerase mRNA

1990 • 55 citations

Reduced mRNA and a nonsense mutation in the insulin-receptor gene produce heritable severe insulin resistance.

1992 • 53 citations

Translation affects immunoglobulin mRNA stability

1989 • 52 citations

Defective splicing of mRNA from one COL1A1 allele of type I collagen in nondeforming (type I) osteogenesis imperfecta.

1993 • 51 citations

Nonsense but Not Missense Mutations Can Decrease the Abundance of Nuclear mRNA for the Mouse Major Urinary Protein, While Both Types of Mutations Can Facilitate Exon Skipping

1994 • 50 citations

T Cell Receptor-β mRNA Splicing: Regulation of Unusual Splicing Intermediates

1993 • 46 citations

Mammalian Nonsense Codons Can Be cis Effectors of Nuclear mRNA Half-Life

1994 • 44 citations

Affinity maturation leads to differential expression of multiple copies of a κ light-chain transgene

1993 • 43 citations

Rous sarcoma virus RNA stability requires an open reading frame in the gag gene and sequences downstream of the gag-pol junction.

1994 • 42 citations

A 5′ exoribonuclease from cytoplasmic extracts of mouse sarcoma 180 ascites cells

1993 • 39 citations

Turnover of mRNA in prokaryotes and lower eukaryotes

1992 • 39 citations

On the linkage between RNA processing and RNA translatability.

1994 • 38 citations

Order of Intron Removal during Splicing of Endogenous Adenine Phosphoribosyltransferase and Dihydrofolate Reductase Pre-mRNA

1993 • 37 citations

Frameshift Mutations in the v-src Gene of Avian Sarcoma Virus Act in cis To Specifically Reduce v-src mRNA Levels

1994 • 36 citations

Evidence for degradation of mRNA encoding alpha-L-iduronidase in Hurler fibroblasts with premature termination alleles.

1994 • 35 citations

Nonsense Codons within the Rous Sarcoma Virus gag Gene Decrease the Stability of Unspliced Viral RNA

1991 • 32 citations

Initiation of Translation Can Occur Only in a Restricted Region of the CYC1 mRNA of Saccharomyces cerevisiae

1995 • 31 citations

T cell receptor-beta mRNA splicing during thymic maturation in vivo and in an inducible T cell clone in vitro.

1993 • 28 citations

Normal transcription of the beta-hexosaminidase alpha-chain gene in the Ashkenazi Tay-Sachs mutation.

1988 • 27 citations

A β°-thalassemic β-globin RNA that is labile in bone marrow cells is relatively stable in HeLa cells

1985 • 27 citations

The molecular basis of HEXA mRNA deficiency caused by the most common Tay-Sachs disease mutation.

1995 • 24 citations

A deletion/insertion mutation in theIDUA gene in a Libyan Jewish patient with Hurler syndrome (mucopolysaeeharidosis IH)

1993 • 24 citations

Nonsense-Mediated mRNA Decay in Xenopus Oocytes and Embryos

1994 • 23 citations

Rous Sarcoma Virus RNA Stability Requires an Open Reading Frame in the gag Gene and Sequences Downstream of the gag-pol Junction

1994 • 22 citations

Complex regulation of the T cell receptor α gene: three different modes of triggering induction

1988 • 14 citations

Expression of the beta-hexosaminidase alpha subunit gene with the four-base insertion of infantile Jewish Tay-Sachs disease

1991 • 13 citations

RNA processing mutants at the dihydrofolate reductase locus in Chinese hamster ovary cells.

1990 • 11 citations