当前位置： SCI文献检索 > PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA期刊下所有文献 > Rescuing Z+ agrin splicing in Nova null mice restores synapse formation and unmasks a physiologic defect in motor neuron firing.

Rescuing Z+ agrin splicing in Nova null mice restores synapse formation and unmasks a physiologic defect in motor neuron firing.

Abstract：

:Synapse formation at the neuromuscular junction (NMJ) requires an alternatively spliced variant of agrin (Z(+) agrin) that is produced only by neurons. Here, we show that Nova1 and Nova2, neuron-specific splicing factors identified as targets in autoimmune motor disease, are essential regulators of Z(+) agrin. Nova1/Nova2 double knockout mice are paralyzed and fail to cluster AChRs at the NMJ, and breeding them with transgenic mice constitutively expressing Z(+) agrin in motor neurons rescued AChR clustering. Surprisingly, however, these rescued mice remained paralyzed, while electrophysiologic studies demonstrated that the motor axon and synapse were functional-spontaneous and evoked recordings revealed synaptic transmission and muscle contraction. These results point to a proximal defect in motor neuron firing in the absence of Nova and reveal a previously unsuspected role for RNA regulation in the physiologic activation of motor neurons.

journal_name

Proc Natl Acad Sci U S A

journal_title

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

authors

Ruggiu M,Herbst R,Kim N,Jevsek M,Fak JJ,Mann MA,Fischbach G,Burden SJ,Darnell RB

doi

10.1073/pnas.0813112106

subject

Has Abstract

pub_date

2009-03-03 00:00:00

pages

3513-8

issue

9

eissn

0027-8424

issn

1091-6490

pii

0813112106

journal_volume

106

pub_type

杂志文章

相关文献

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA文献大全
  • Quantifying and mapping the human appropriation of net primary production in earth's terrestrial ecosystems.

    abstract：:Human appropriation of net primary production (HANPP), the aggregate impact of land use on biomass available each year in ecosystems, is a prominent measure of the human domination of the biosphere. We present a comprehensive assessment of global HANPP based on vegetation modeling, agricultural and forestry statistics...

    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.0704243104

    authors： Haberl H,Erb KH,Krausmann F,Gaube V,Bondeau A,Plutzar C,Gingrich S,Lucht W,Fischer-Kowalski M

    更新日期：2007-07-31 00:00:00

  • Loss of ephrin-A5 function disrupts lens fiber cell packing and leads to cataract.

    abstract：:Cell-cell interactions organize lens fiber cells into highly ordered structures to maintain transparency. However, signals regulating such interactions have not been well characterized. We report here that ephrin-A5, a ligand of the Eph receptor tyrosine kinases, plays a key role in lens fiber cell shape and cell-cell...

    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.0808987105

    authors： Cooper MA,Son AI,Komlos D,Sun Y,Kleiman NJ,Zhou R

    更新日期：2008-10-28 00:00:00

  • Polyamines protect Escherichia coli cells from the toxic effect of oxygen.

    abstract：:Wild-type Escherichia coli cells grow normally in 95% O(2)/5% CO(2). In contrast, cells that cannot make polyamines because of mutations in the biosynthetic pathway are rapidly killed by incubation in 95% O(2)/5% CO(2). Addition of polyamines prevents the toxic effect of oxygen, permitting cell survival and optimal gr...

    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.2627990100

    authors： Chattopadhyay MK,Tabor CW,Tabor H

    更新日期：2003-03-04 00:00:00

  • Cytochrome b5 potentiation of cytochrome P-450 catalytic activity demonstrated by a vaccinia virus-mediated in situ reconstitution system.

    abstract：:A cDNA containing the full coding region of human cytochrome b5 was inserted into a vaccinia virus cDNA expression vector. Infection of human thymidine kinase-minus (TK-) 143 cells in culture with this recombinant virus resulted in production of 0.3 nmol of cytochrome b5 per mg of cell lysate protein. The expressed cy...

    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.87.14.5425

    authors： Aoyama T,Nagata K,Yamazoe Y,Kato R,Matsunaga E,Gelboin HV,Gonzalez FJ

    更新日期：1990-07-01 00:00:00

  • Stimulation of release of adrenal catecholamine by adenosine 3':5'-cyclic monophosphate and theophylline in the absence of extracellular Ca 2+ .

    abstract：:Stimulation of catecholamine release was studied in the isolated adrenal of the cat during retrograde perfusion. Theophylline, adenosine 3':5'-cyclic monophosphate (cyclic AMP), and dibutyryl-cyclic AMP stimulated catecholamine release in adrenal chromaffin tissue; adenosine, 2'-AMP, 3'-AMP, and 5'-AMP were ineffectiv...

    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.69.4.834

    authors： Peach MJ

    更新日期：1972-04-01 00:00:00

  • Copper- and copper-N-heterocyclic carbene-catalyzed C-H activating carboxylation of terminal alkynes with CO2 at ambient conditions.

    abstract：:The use of carbon dioxide as a renewable and environmentally friendly source of carbon in organic synthesis is a highly attractive approach, but its real world applications remain a great challenge. The major obstacles for commercialization of most current protocols are their low catalytic performances, harsh reaction...

    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.1010962107

    authors： Yu D,Zhang Y

    更新日期：2010-11-23 00:00:00

  • Room-temperature single-electron junction.

    abstract：:The design, realization, and test performances of an electronic junction based on single-electron phenomena that works in the air at room temperature are hereby reported. The element consists of an electrochemically etched sharp tungsten stylus over whose tip a nanometer-size crystal was synthesized. Langmuir-Blodgett...

    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.93.20.10556

    authors： Facci P,Erokhin V,Carrara S,Nicolini C

    更新日期：1996-10-01 00:00:00

  • Functionally active targeting domain of the beta-adrenergic receptor kinase: an inhibitor of G beta gamma-mediated stimulation of type II adenylyl cyclase.

    abstract：:The beta-adrenergic receptor kinase (beta ARK) phosphorylates its membrane-associated receptor substrates, such as the beta-adrenergic receptor, triggering events leading to receptor desensitization. beta ARK activity is markedly stimulated by the isoprenylated beta gamma subunit complex of heterotrimeric guanine nucl...

    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.91.9.3637

    authors： Inglese J,Luttrell LM,Iñiguez-Lluhi JA,Touhara K,Koch WJ,Lefkowitz RJ

    更新日期：1994-04-26 00:00:00

  • Glutathione-dependent and -independent oxidative stress-control mechanisms distinguish normal human mammary epithelial cell subsets.

    abstract：:Mechanisms that control the levels and activities of reactive oxygen species (ROS) in normal human mammary cells are poorly understood. We show that purified normal human basal mammary epithelial cells maintain low levels of ROS primarily by a glutathione-dependent but inefficient antioxidant mechanism that uses mitoc...

    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.1403813111

    authors： Kannan N,Nguyen LV,Makarem M,Dong Y,Shih K,Eirew P,Raouf A,Emerman JT,Eaves CJ

    更新日期：2014-05-27 00:00:00

  • A biochemical approach to identifying microRNA targets.

    abstract：:Identifying the downstream targets of microRNAs (miRNAs) is essential to understanding cellular regulatory networks. We devised a direct biochemical method for miRNA target discovery that combined RNA-induced silencing complex (RISC) purification with microarray analysis of bound mRNAs. Because targets of miR-124a hav...

    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.0709971104

    authors： Karginov FV,Conaco C,Xuan Z,Schmidt BH,Parker JS,Mandel G,Hannon GJ

    更新日期：2007-12-04 00:00:00

  • Bursting electrical activity in pancreatic beta cells caused by Ca(2+)- and voltage-inactivated Ca2+ channels.

    abstract：:We investigate the hypothesis that two classes of Ca2+ currents, one quickly inactivated by Ca2+ and one slowly inactivated by voltage, contribute to bursting electrical activity in pancreatic islets. A mathematical model of these currents is fit to the experimental whole-cell current-voltage and inactivation profiles...

    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.88.9.3897

    authors： Keizer J,Smolen P

    更新日期：1991-05-01 00:00:00

  • Engineering a BCR-ABL-activated caspase for the selective elimination of leukemic cells.

    abstract：:Increased understanding of the precise molecular mechanisms involved in cell survival and cell death signaling pathways offers the promise of harnessing these molecules to eliminate cancer cells without damaging normal cells. Tyrosine kinase oncoproteins promote the genesis of leukemias through both increased cell pro...

    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.1206551110

    authors： Kurokawa M,Ito T,Yang CS,Zhao C,Macintyre AN,Rizzieri DA,Rathmell JC,Deininger MW,Reya T,Kornbluth S

    更新日期：2013-02-05 00:00:00

  • A network analysis of committees in the U.S. House of Representatives.

    abstract：:Network theory provides a powerful tool for the representation and analysis of complex systems of interacting agents. Here, we investigate the U.S. House of Representatives network of committees and subcommittees, with committees connected according to "interlocks," or common membership. Analysis of this network revea...

    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.0500191102

    authors： Porter MA,Mucha PJ,Newman ME,Warmbrand CM

    更新日期：2005-05-17 00:00:00

  • Identification of a region of beta 2-glycoprotein I critical for lipid binding and anti-cardiolipin antibody cofactor activity.

    abstract：:beta 2-Glycoprotein I (beta 2-GPI), a phospholipid-binding plasma protein, is an absolute requirement (cofactor) for the binding of autoimmune-type anti-cardiolipin (aCL) antibodies to cardiolipin (CL). The nature of this cofactor activity and the specific regions of the molecule involved have not yet been determined....

    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.90.6.2141

    authors： Hunt JE,Simpson RJ,Krilis SA

    更新日期：1993-03-15 00:00:00

  • Projected impacts of climate change on salmon habitat restoration.

    abstract：:Throughout the world, efforts are under way to restore watersheds, but restoration planning rarely accounts for future climate change. Using a series of linked models of climate, land cover, hydrology, and salmon population dynamics, we investigated the impacts of climate change on the effectiveness of proposed habita...

    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.0701685104

    authors： Battin J,Wiley MW,Ruckelshaus MH,Palmer RN,Korb E,Bartz KK,Imaki H

    更新日期：2007-04-17 00:00:00

  • Crystal structure of thermostable DNA photolyase: pyrimidine-dimer recognition mechanism.

    abstract：:DNA photolyase is a pyrimidine-dimer repair enzyme that uses visible light. Photolyase generally contains two chromophore cofactors. One is a catalytic cofactor directly contributing to the repair of a pyrimidine-dimer. The other is a light-harvesting cofactor, which absorbs visible light and transfers energy to the 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.241371398

    authors： Komori H,Masui R,Kuramitsu S,Yokoyama S,Shibata T,Inoue Y,Miki K

    更新日期：2001-11-20 00:00:00

  • Whirly proteins maintain plastid genome stability in Arabidopsis.

    abstract：:Maintenance of genome stability is essential for the accurate propagation of genetic information and cell growth and survival. Organisms have therefore developed efficient strategies to prevent DNA lesions and rearrangements. Much of the information concerning these strategies has been obtained through the study of ba...

    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.0901710106

    authors： Maréchal A,Parent JS,Véronneau-Lafortune F,Joyeux A,Lang BF,Brisson N

    更新日期：2009-08-25 00:00:00

  • IA-2beta, but not IA-2, is induced by ghrelin and inhibits glucose-stimulated insulin secretion.

    abstract：:Ghrelin is a newly discovered peptide and an endogenous ligand for growth hormone (GH) secretagogue (GHS) receptor. It has been shown to possess various central and peripheral effects, including GH secretion, food intake, and gastric and cardiac effects. Ghrelin and the GHS receptor are expressed also in pancreatic is...

    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.0502470102

    authors： Doi A,Shono T,Nishi M,Furuta H,Sasaki H,Nanjo K

    更新日期：2006-01-24 00:00:00

  • Visual motion perception.

    abstract：:The primate visual motion system performs numerous functions essential for survival in a dynamic visual world. Prominent among these functions is the ability to recover and represent the trajectories of objects in a form that facilitates behavioral responses to those movements. The first step toward this goal, which c...

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

    pub_type： 杂志文章,评审

    doi：10.1073/pnas.92.7.2433

    authors： Albright TD,Stoner GR

    更新日期：1995-03-28 00:00:00

  • Maintenance of an extrachromosomal plasmid vector in mouse embryonic stem cells.

    abstract：:We have constructed and characterized a polyoma virus-based plasmid that is maintained as an autonomously replicating extrachromosomal element (episome) in mouse embryonic stem (ES) cells. Plasmid pMGD20neo contains the polyoma origin of replication harboring a mutated enhancer (PyF101), a modified polyoma early regio...

    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.92.5.1292

    authors： Gassmann M,Donoho G,Berg P

    更新日期：1995-02-28 00:00:00

  • Power spectra reveal the influence of stochasticity on nonlinear population dynamics.

    abstract：:Stochasticity alters the nonlinear dynamics of inherently cycling populations. The power spectrum can describe and explain the impacts of stochasticity. We fitted models to short observed time series of flour beetle populations in the frequency domain, then used a well fitting stochastic mechanistic model to generate ...

    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.0608571103

    authors： Reuman DC,Desharnais RA,Costantino RF,Ahmad OS,Cohen JE

    更新日期：2006-12-05 00:00:00

  • Kinetic evidence for a ligand-binding-induced conformational transition in the T cell receptor.

    abstract：:Thermodynamics and kinetics of the interaction between T cell receptor specific for cytomegalovirus peptide (TCR(CMV)) and its specific ligand, pp65-HLA-A*0201 complex, were studied by surface plasmon resonance and stopped-flow methods. In the latter measurements, fluorescence resonance energy transfer (FRET) between ...

    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.0707061104

    authors： Gakamsky DM,Lewitzki E,Grell E,Saulquin X,Malissen B,Montero-Julian F,Bonneville M,Pecht I

    更新日期：2007-10-16 00:00:00

  • Photochemical electron injection into redox-active proteins.

    abstract：:A new method is presented that makes it possible to inject electrons rapidly into redox-active proteins by means of a short light flash. Reduced carboxymethylated cytochrome c (CmCyt c) with carbon monoxide bound to the heme iron is mixed with the oxidized acceptor protein. Upon rapid photodissociation of CO the appar...

    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.94.12.6176

    authors： Brzezinski P,Wilson MT

    更新日期：1997-06-10 00:00:00

  • The gametic central cell of Arabidopsis determines the lifespan of adjacent accessory cells.

    abstract：:Plant germ cells develop in specialized haploid structures, termed gametophytes. The female gametophyte patterns of flowering plants are diverse, with often unknown adaptive value. Here we present the Arabidopsis fiona mutant, which forms a female gametophyte that is structurally and functionally reminiscent of a phyl...

    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.1012795108

    authors： Kägi C,Baumann N,Nielsen N,Stierhof YD,Gross-Hardt R

    更新日期：2010-12-21 00:00:00

  • Allergen- and bacterial antigen-specific T-cell clones established from atopic donors show a different profile of cytokine production.

    abstract：:We have established a large panel of T-cell clones (TCCs) specific for Dermatophagoides pteronyssinus and Lolium perenne group I grass pollen allergens (total, 61) and for tetanus toxoid and protein purified derivative bacterial antigens (total, 38) from the peripheral blood of two atopic individuals and then analyzed...

    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.88.10.4538

    authors： Parronchi P,Macchia D,Piccinni MP,Biswas P,Simonelli C,Maggi E,Ricci M,Ansari AA,Romagnani S

    更新日期：1991-05-15 00:00:00

  • Accelerated induction of experimental allergic encephalomyelitis in PL/J mice by a non-V beta 8-specific superantigen.

    abstract：:Superantigens such as the staphylococcal enterotoxins can play an important role in exacerbation of autoimmune disorders such as experimental allergic encephalomyelitis (EAE) in mice. In fact, superantigens can reactivate EAE in PL/J mice that have been sensitized to rat myelin basic protein (MBP). The T-cell subset p...

    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.92.13.6082

    authors： Soos JM,Hobeika AC,Butfiloski EJ,Schiffenbauer J,Johnson HM

    更新日期：1995-06-20 00:00:00

  • The first day of the Cenozoic.

    abstract：:Highly expanded Cretaceous-Paleogene (K-Pg) boundary section from the Chicxulub peak ring, recovered by International Ocean Discovery Program (IODP)-International Continental Scientific Drilling Program (ICDP) Expedition 364, provides an unprecedented window into the immediate aftermath of the impact. Site M0077 inclu...

    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.1909479116

    authors： Gulick SPS,Bralower TJ,Ormö J,Hall B,Grice K,Schaefer B,Lyons S,Freeman KH,Morgan JV,Artemieva N,Kaskes P,de Graaff SJ,Whalen MT,Collins GS,Tikoo SM,Verhagen C,Christeson GL,Claeys P,Coolen MJL,Goderis S,Goto K,

    更新日期：2019-09-24 00:00:00

  • Local kinetics of morphogen gradients.

    abstract：:Some aspects of pattern formation in developing embryos can be described by nonlinear reaction-diffusion equations. An important class of these models accounts for diffusion and degradation of a locally produced single chemical species. At long times, solutions of such models approach a steady state in which the conce...

    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.1019245108

    authors： Gordon PV,Sample C,Berezhkovskii AM,Muratov CB,Shvartsman SY

    更新日期：2011-04-12 00:00:00

  • Intrinsic and extrinsic contributions to stochasticity in gene expression.

    abstract：:Gene expression is a stochastic, or "noisy," process. This noise comes about in two ways. The inherent stochasticity of biochemical processes such as transcription and translation generates "intrinsic" noise. In addition, fluctuations in the amounts or states of other cellular components lead indirectly to variation i...

    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.162041399

    authors： Swain PS,Elowitz MB,Siggia ED

    更新日期：2002-10-01 00:00:00

  • Transcription of cloned tRNA gene fragments and subfragments injected into the oocyte nucleus of Xenopus laevis.

    abstract：:Cloned 3.18 kilobase fragments of Xenopus laevis DNA containing genes for tRNAMet1 and for at least one other 4S RNA species are transcribed rapidly after their injection into the nucleus of X. laevis oocytes. The newly synthesized RNA can be resolved by gel electrophoresis into a few predominant 4S RNA species and 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.75.3.1176

    authors： Kressmann A,Clarkson SG,Pirrotta V,Birnstiel ML

    更新日期：1978-03-01 00:00:00