Quality control of assembly-defective U1 snRNAs by decapping and 5'-to-3' exonucleolytic digestion.

Abstract:

:The accurate biogenesis of RNA-protein complexes is a key aspect of eukaryotic cells. Defects in Sm protein complex binding to snRNAs are known to reduce levels of snRNAs, suggesting an unknown quality control system for small nuclear ribonucleoprotein (snRNP) assembly. snRNA quality control may also be relevant in spinal muscular atrophy, which is caused by defects in the survival motor neuron (SMN)1 gene, an assembly factor for loading the Sm complex on snRNAs and, when severely reduced, can lead to reduced levels of snRNAs and splicing defects. To determine how assembly-defective snRNAs are degraded, we first demonstrate that yeast U1 Sm-mutant snRNAs are degraded either by Rrp6- or by Dcp2-dependent decapping/5'-to-3' decay. Knockdown of the decapping enzyme DCP2 in mammalian cells also increases the levels of assembly-defective snRNAs and suppresses some splicing defects seen in SMN-deficient cells. These results identify a conserved mechanism of snRNA quality control, and also suggest a general paradigm wherein the phenotype of an "RNP assembly disease" might be suppressed by inhibition of a competing RNA quality control mechanism.

authors

Shukla S,Parker R

doi

10.1073/pnas.1412614111

subject

Has Abstract

pub_date

2014-08-12 00:00:00

pages

E3277-86

issue

32

eissn

0027-8424

issn

1091-6490

pii

1412614111

journal_volume

111

pub_type

杂志文章
  • Biphasic regulation by dibutyryl cyclic AMP of tubulin and actin mRNA levels in neuroblastoma cells.

    abstract::Blot hybridization analysis that used labeled tubulin cDNA probes revealed that N6,O2'-dibutyryladenosine 3',5'-cyclic monophosphate [dibutyryl cyclic AMP (Bt2cAMP)] initially increases and later decreases the level of tubulin mRNA in a neuroblastoma-glioma hybrid cell line as well as in the parent cells. A significan...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.80.14.4243

    authors: Ginzburg I,Rybak S,Kimhi Y,Littauer UZ

    更新日期:1983-07-01 00:00:00

  • Molecular dissection of the evolution of carbapenem-resistant multilocus sequence type 258 Klebsiella pneumoniae.

    abstract::Infections caused by drug-resistant bacteria are a major problem worldwide. Carbapenem-resistant Klebsiella pneumoniae, most notably isolates classified as multilocus sequence type (ST) 258, have emerged as an important cause of hospital deaths. ST258 isolates are predominantly multidrug resistant, and therefore infec...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1321364111

    authors: Deleo FR,Chen L,Porcella SF,Martens CA,Kobayashi SD,Porter AR,Chavda KD,Jacobs MR,Mathema B,Olsen RJ,Bonomo RA,Musser JM,Kreiswirth BN

    更新日期:2014-04-01 00:00:00

  • Membrane sidedness and the interaction of H+ and K+ on Ca2(+)-activated K+ transport in human red blood cells.

    abstract::The sided effects of H+ on Ca2(+)-stimulated K+ transport (the Gardos channel) were studied in human red blood cells. Cells were loaded with Ca2+ during energy depletion with the internal pH adjusted to desired levels prior to treatment with the anion-exchange inhibitor 4,4'-diisothiocyanostilbene-2,2'-disulfonic acid...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.87.5.1998

    authors: Heinz A,Hoffman JF

    更新日期:1990-03-01 00:00:00

  • In vitro synthesis of RNA that contains polyadenylate by virion-associated RNA polymerase of vesicular stomatitis virus.

    abstract::The RNA synthesized in vitro by the virion-associated RNA-instructed RNA polymerase of purified vesicular stomatitis virus contains polyadenylate sequences. These have been demonstrated by their partial resistance to pancreatic and T(1) ribonucleases and their capacity to bind to poly(U) filters and oligo(dT)-cellulos...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.70.12.3566

    authors: Banerjee AK,Rhodes DP

    更新日期:1973-12-01 00:00:00

  • Quantitative expression profiling of identified neurons reveals cell-specific constraints on highly variable levels of gene expression.

    abstract::The postdevelopmental basis of cellular identity and the unique cellular output of a particular neuron type are of particular interest in the nervous system because a detailed understanding of circuits responsible for complex processes in the brain is impeded by the often ambiguous classification of neurons in these c...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.0705827104

    authors: Schulz DJ,Goaillard JM,Marder EE

    更新日期:2007-08-07 00:00:00

  • Genetic variation in putative regulatory loci controlling gene expression in breast cancer.

    abstract::Candidate single-nucleotide polymorphisms (SNPs) were analyzed for associations to an unselected whole genome pool of tumor mRNA transcripts in 50 unrelated patients with breast cancer. SNPs were selected from 203 candidate genes of the reactive oxygen species pathway. We describe a general statistical framework for t...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.0601893103

    authors: Kristensen VN,Edvardsen H,Tsalenko A,Nordgard SH,Sørlie T,Sharan R,Vailaya A,Ben-Dor A,Lønning PE,Lien S,Omholt S,Syvänen AC,Yakhini Z,Børresen-Dale AL

    更新日期:2006-05-16 00:00:00

  • Human-modified landscapes alter mammal resource and habitat use and trophic structure.

    abstract::The broad negative consequences of habitat degradation on biodiversity have been studied, but the complex effects of natural-agricultural landscape matrices remain poorly understood. Here we used stable carbon and nitrogen isotopes to detect changes in mammal resource and habitat use and trophic structure between pres...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1904384116

    authors: Magioli M,Moreira MZ,Fonseca RCB,Ribeiro MC,Rodrigues MG,Ferraz KMPMB

    更新日期:2019-09-10 00:00:00

  • The primary structure of sensory rhodopsin II: a member of an additional retinal protein subgroup is coexpressed with its transducer, the halobacterial transducer of rhodopsin II.

    abstract::The blue-light receptor genes (sopII) of sensory rhodopsin (SR) II were cloned from two species, the halophilic bacteria Haloarcula vallismortis (vSR-II) and Natronobacterium pharaonis (pSR-II). Upstream of both sopII gene loci, sequences corresponding to the halobacterial transducer of rhodopsin (Htr) II were recogni...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.92.7.3036

    authors: Seidel R,Scharf B,Gautel M,Kleine K,Oesterhelt D,Engelhard M

    更新日期:1995-03-28 00:00:00

  • Microglial glucocorticoid receptors play a pivotal role in regulating dopaminergic neurodegeneration in parkinsonism.

    abstract::Among the pathogenic processes contributing to dopaminergic neuron (DN) death in Parkinson disease (PD), evidence points to non-cell-autonomous mechanisms, particularly chronic inflammation mounted by activated microglia. Yet little is known about endogenous regulatory processes that determine microglial actions in pa...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1017820108

    authors: Ros-Bernal F,Hunot S,Herrero MT,Parnadeau S,Corvol JC,Lu L,Alvarez-Fischer D,Carrillo-de Sauvage MA,Saurini F,Coussieu C,Kinugawa K,Prigent A,Höglinger G,Hamon M,Tronche F,Hirsch EC,Vyas S

    更新日期:2011-04-19 00:00:00

  • Structural characterization of the alpha-glycerol-3-phosphate dehydrogenase-encoding gene of Drosophila melanogaster.

    abstract::In Drosophila, multiple isoforms of alpha-glycerol-3-phosphate dehydrogenase (sn-glycerol-3-phosphate: NAD+ 2-oxidoreductase, EC 1.1.1.8) are produced in a tissue- and stage-specific manner. To understand the underlying molecular basis of these isoforms, we have sequenced a 5.8-kilobase region of the Drosophila genome...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.86.13.5020

    authors: von Kalm L,Weaver J,DeMarco J,MacIntyre RJ,Sullivan DT

    更新日期:1989-07-01 00:00:00

  • Working memory capacity predicts individual differences in social-distancing compliance during the COVID-19 pandemic in the United States.

    abstract::Noncompliance with social distancing during the early stage of the coronavirus disease 2019 (COVID-19) pandemic poses a great challenge to the public health system. These noncompliance behaviors partly reflect people's concerns for the inherent costs of social distancing while discounting its public health benefits. W...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.2008868117

    authors: Xie W,Campbell S,Zhang W

    更新日期:2020-07-28 00:00:00

  • Active transport of calcium in inverted membrane vesicles of Escherichia coli.

    abstract::Accumulation of (45)Ca(++) was found to occur in membrane vesicles of E. coli prepared by lysis with a French pressure cell. The uptake occurs by active transport, requiring an energy source. Substrates of the electron transport chain, including D-lactate, reduced phenazine methosulfate, and NADH, stimulated accumulat...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.71.12.5042

    authors: Rosen BP,McClees JS

    更新日期:1974-12-01 00:00:00

  • Harmonic dynamics of the abelian sandpile.

    abstract::The abelian sandpile is a cellular automaton which serves as the archetypical model to study self-organized criticality, a phenomenon occurring in various biological, physical, and social processes. Its recurrent configurations form an abelian group, whose identity is a fractal composed of self-similar patches. Here, ...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1812015116

    authors: Lang M,Shkolnikov M

    更新日期:2019-02-19 00:00:00

  • NADPH oxidase mediates oxidative stress in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine model of Parkinson's disease.

    abstract::Parkinson's disease (PD) is a neurodegenerative disorder of uncertain pathogenesis characterized by a loss of substantia nigra pars compacta (SNpc) dopaminergic (DA) neurons, and can be modeled by the neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). Both inflammatory processes and oxidative stress may c...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.0937239100

    authors: Wu DC,Teismann P,Tieu K,Vila M,Jackson-Lewis V,Ischiropoulos H,Przedborski S

    更新日期:2003-05-13 00:00:00

  • Reducible crosslinks in hydroxylysine-deficient collagens of a heritable disorder of connective tissue.

    abstract::Reducible compounds that participate in crosslinking were analyzed in hydroxylysine-deficient collagens of patients with a heritable disorder of connective tissue. After treatment with [(3)H]sodium borohydride, new compounds, as well as a totally different pattern of tritiated compounds, were found in hydroxylysine-de...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.69.9.2594

    authors: Eyre DR,Glimcher MJ

    更新日期:1972-09-01 00:00:00

  • Structural basis for gating mechanisms of a eukaryotic P-glycoprotein homolog.

    abstract::P-glycoprotein is an ATP-binding cassette multidrug transporter that actively transports chemically diverse substrates across the lipid bilayer. The precise molecular mechanism underlying transport is not fully understood. Here, we present crystal structures of a eukaryotic P-glycoprotein homolog, CmABCB1 from Cyanidi...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1321562111

    authors: Kodan A,Yamaguchi T,Nakatsu T,Sakiyama K,Hipolito CJ,Fujioka A,Hirokane R,Ikeguchi K,Watanabe B,Hiratake J,Kimura Y,Suga H,Ueda K,Kato H

    更新日期:2014-03-18 00:00:00

  • How native-state topology affects the folding of dihydrofolate reductase and interleukin-1beta.

    abstract::The overall structure of the transition-state and intermediate ensembles observed experimentally for dihydrofolate reductase and interleukin-1beta can be obtained by using simplified models that have almost no energetic frustration. The predictive power of these models suggests that, even for these very large proteins...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.100547897

    authors: Clementi C,Jennings PA,Onuchic JN

    更新日期:2000-05-23 00:00:00

  • ACHT4-driven oxidation of APS1 attenuates starch synthesis under low light intensity in Arabidopsis plants.

    abstract::The regulatory mechanisms that use signals of low levels of reactive oxygen species (ROS) could be obscured by ROS produced under stress and thus are better investigated under homeostatic conditions. Previous studies showed that the chloroplastic atypical thioredoxin ACHT1 is oxidized by 2-Cys peroxiredoxin (2-Cys Prx...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1515513112

    authors: Eliyahu E,Rog I,Inbal D,Danon A

    更新日期:2015-10-13 00:00:00

  • Huntingtin-associated protein (HAP1): discrete neuronal localizations in the brain resemble those of neuronal nitric oxide synthase.

    abstract::Huntington disease stems from a mutation of the protein huntingtin and is characterized by selective loss of discrete neuronal populations in the brain. Despite a massive loss of neurons in the corpus striatum, NO-generating neurons are intact. We recently identified a brain-specific protein that associates with hunti...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.93.10.4839

    authors: Li XJ,Sharp AH,Li SH,Dawson TM,Snyder SH,Ross CA

    更新日期:1996-05-14 00:00:00

  • Temporal and developmental requirements for the Prader-Willi imprinting center.

    abstract::Imprinted gene expression associated with Prader-Willi syndrome (PWS) and Angelman syndrome (AS) is controlled by two imprinting centers (ICs), the PWS-IC and the AS-IC. The PWS-IC operates in cis to activate transcription of genes that are expressed exclusively from the paternal allele. We have created a conditional ...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1115057109

    authors: DuBose AJ,Smith EY,Johnstone KA,Resnick JL

    更新日期:2012-02-28 00:00:00

  • Pericentromeric satellite repeat expansions through RNA-derived DNA intermediates in cancer.

    abstract::Aberrant transcription of the pericentromeric human satellite II (HSATII) repeat is present in a wide variety of epithelial cancers. In deriving experimental systems to study its deregulation, we observed that HSATII expression is induced in colon cancer cells cultured as xenografts or under nonadherent conditions in ...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1518008112

    authors: Bersani F,Lee E,Kharchenko PV,Xu AW,Liu M,Xega K,MacKenzie OC,Brannigan BW,Wittner BS,Jung H,Ramaswamy S,Park PJ,Maheswaran S,Ting DT,Haber DA

    更新日期:2015-12-08 00:00:00

  • Cowpox virus encodes a protein that binds B7.1 and B7.2 and subverts T cell costimulation.

    abstract::Costimulation is required for optimal T cell activation, yet it is unclear whether poxviruses dedicatedly subvert costimulation during infection. Here, we report that the secreted M2 protein encoded by cowpox virus (CPXV) specifically interacts with human and murine B7.1 (CD80) and B7.2 (CD86). We also show that M2 co...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1909414116

    authors: Wang X,Piersma SJ,Elliott JI,Errico JM,Gainey MD,Yang L,Nelson CA,Yokoyama WM,Fremont DH

    更新日期:2019-10-15 00:00:00

  • Identification of receptors for phorbol ester tumor promoters in intact mammalian cells and of an inhibitor of receptor binding in biologic fluids.

    abstract::Utilizing [3H]phorbol dibutyrate [P(Bu)2], we have developed an assay for high-affinity phorbol ester receptors in intact rat embryo fibroblasts. At 37 degrees C, binding of [3H]P(Bu)2 reached a maximum within 10 min and was rapidly reversible. The tumor promoters 12-O-tetradecanoyl-phorbol 13-acetate, teleocidin B, a...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.78.4.2315

    authors: Horowitz AD,Greenebaum E,Weinstein IB

    更新日期:1981-04-01 00:00:00

  • Multivariate proteomic analysis of murine embryonic stem cell self-renewal versus differentiation signaling.

    abstract::A number of extracellular stimuli, including soluble cytokines and insoluble matrix factors, are known to influence murine embryonic stem cell self-renewal and differentiation behavioral responses via intracellular signaling pathways, but their net effects in combination are difficult to understand. To gain insight co...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.0308768101

    authors: Prudhomme W,Daley GQ,Zandstra P,Lauffenburger DA

    更新日期:2004-03-02 00:00:00

  • Germ-line mutations of the APC gene in 53 familial adenomatous polyposis patients.

    abstract::We searched for germ-line mutations of the APC gene in 79 unrelated patients with familial adenomatous polyposis using a ribonuclease protection analysis coupled with polymerase chain reaction amplifications of genomic DNA. Mutations were found in 53 patients (67%); 28 of the mutations were small deletions and 2 were ...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.89.10.4452

    authors: Miyoshi Y,Ando H,Nagase H,Nishisho I,Horii A,Miki Y,Mori T,Utsunomiya J,Baba S,Petersen G

    更新日期:1992-05-15 00:00:00

  • Equitable decision making is associated with neural markers of intrinsic value.

    abstract::Standard economic and evolutionary models assume that humans are fundamentally selfish. On this view, any acts of prosociality--such as cooperation, giving, and other forms of altruism--result from covert attempts to avoid social injunctions against selfishness. However, even in the absence of social pressure, individ...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章,随机对照试验

    doi:10.1073/pnas.1112324108

    authors: Zaki J,Mitchell JP

    更新日期:2011-12-06 00:00:00

  • L-acetylcarnitine causes rapid antidepressant effects through the epigenetic induction of mGlu2 receptors.

    abstract::Epigenetic mechanisms are involved in the pathophysiology of depressive disorders and are unique potential targets for therapeutic intervention. The acetylating agent L-acetylcarnitine (LAC), a well-tolerated drug, behaves as an antidepressant by the epigenetic regulation of type 2 metabotropic glutamate (mGlu2) recep...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1216100110

    authors: Nasca C,Xenos D,Barone Y,Caruso A,Scaccianoce S,Matrisciano F,Battaglia G,Mathé AA,Pittaluga A,Lionetto L,Simmaco M,Nicoletti F

    更新日期:2013-03-19 00:00:00

  • Selective toxicity of diphtheria toxin for malignant cells.

    abstract::Purified diphtheria toxin is shown to inhibit protein synthesis in Ehrlich-Lettré ascites carcinoma cells in vitro. Protein synthesis in Ehrlich-Lettré cells is at least 10,000 times more sensitive to toxin than protein synthesis in normal mouse spleen or thymus cells. This sensitivity correlates with the observation ...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.71.7.2707

    authors: Iglewski BH,Rittenberg MB

    更新日期:1974-07-01 00:00:00

  • Nuclear magnetic resonance spectroscopy: 13C spectra of some common nucleotides.

    abstract::Natural-abundance (13)C nuclear magnetic resonance spectra of ten common nucleotides in neutral, aqueous solutions have been determined and interpreted. The spectra of two of these substances were also determined in acidic solutions, and several of the carbon chemical shifts were found to depend markedly on pH. Within...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.65.1.19

    authors: Dorman DE,Roberts JD

    更新日期:1970-01-01 00:00:00

  • Pleckstrin homology domain-interacting protein (PHIP) as a marker and mediator of melanoma metastasis.

    abstract::Although melanomas with mutant v-Raf murine sarcoma viral oncogene homolog B1 (BRAF) can now be effectively targeted, there is no molecular target for most melanomas expressing wild-type BRAF. Here, we show that the activation of Pleckstrin homology domain-interacting protein (PHIP), promotes melanoma metastasis, can ...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1119949109

    authors: De Semir D,Nosrati M,Bezrookove V,Dar AA,Federman S,Bienvenu G,Venna S,Rangel J,Climent J,Meyer Tamgüney TM,Thummala S,Tong S,Leong SP,Haqq C,Billings P,Miller JR 3rd,Sagebiel RW,Debs R,Kashani-Sabet M

    更新日期:2012-05-01 00:00:00