Inhibition of DUX4 expression with antisense LNA gapmers as a therapy for facioscapulohumeral muscular dystrophy.

abstract：:Here, we describe the identification and characterization of a nuclear body (matrix-associated deacetylase body) whose formation and integrity depend on deacetylase activity. Typically, there are 20-40 0.5-microM bodies per nucleus, although the size and number can vary substantially. The structure appears to contain ...

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

authors： Downes M,Ordentlich P,Kao HY,Alvarez JG,Evans RM

更新日期：2000-09-12 00:00:00

abstract：:Some of the most important impacts of global climate change will be felt among the populations, predominantly in developing countries, referred to as "subsistence" or "smallholder" farmers. Their vulnerability to climate change comes both from being predominantly located in the tropics, and from various socioeconomic,...

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

authors： Morton JF

更新日期：2007-12-11 00:00:00

abstract：:Pentameric ligand-gated ion channels (pLGICs), which mediate chemo-electric signal transduction in animals, have been recently found in bacteria. Despite clear sequence and 3D structure homology, the phylogenetic distance between prokaryotic and eukaryotic homologs suggests significant structural divergences, especial...

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

authors： Duret G,Van Renterghem C,Weng Y,Prevost M,Moraga-Cid G,Huon C,Sonner JM,Corringer PJ

更新日期：2011-07-19 00:00:00

abstract：:Hyperapobetalipoproteinemia is defined as the combination of a normal low density lipoprotein (LDL) cholesterol in the face of an increased LDL apolipoprotein B (apoB) protein. To examine the physical basis for the apparent disproportion between LDL cholesterol and apoB characteristic of this syndrome, we used density...

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

authors： Teng B,Thompson GR,Sniderman AD,Forte TM,Krauss RM,Kwiterovich PO Jr

更新日期：1983-11-01 00:00:00

abstract：:Chorismate mutase acts at the first branch-point of aromatic amino acid biosynthesis and catalyzes the conversion of chorismate to prephenate. The results of molecular dynamics simulations of the substrate in solution and in the active site of chorismate mutase are reported. Two nonreactive conformers of chorismate ar...

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

authors： Guo H,Cui Q,Lipscomb WN,Karplus M

更新日期：2001-07-31 00:00:00

abstract：:A human liver cDNA library was screened by colony hybridization with a bovine factor X cDNA probe. Three of the positive plasmids contained overlapping DNA that coded for most of human factor X mRNA. DNA sequence analysis of these three clones allowed the prediction of the complete amino acid sequence of plasma factor...

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

authors： Fung MR,Hay CW,MacGillivray RT

更新日期：1985-06-01 00:00:00

abstract：:Interstitial telomeric sequences (ITSs) are present in many eukaryotic genomes and are linked to genome instabilities and disease in humans. The mechanisms responsible for ITS-mediated genome instability are not understood in molecular detail. Here, we use a model Saccharomyces cerevisiae system to characterize genome...

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

authors： Aksenova AY,Greenwell PW,Dominska M,Shishkin AA,Kim JC,Petes TD,Mirkin SM

更新日期：2013-12-03 00:00:00

abstract：:Brown-rot fungi such as Postia placenta are common inhabitants of forest ecosystems and are also largely responsible for the destructive decay of wooden structures. Rapid depolymerization of cellulose is a distinguishing feature of brown-rot, but the biochemical mechanisms and underlying genetics are poorly understood...

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

authors： Martinez D,Challacombe J,Morgenstern I,Hibbett D,Schmoll M,Kubicek CP,Ferreira P,Ruiz-Duenas FJ,Martinez AT,Kersten P,Hammel KE,Vanden Wymelenberg A,Gaskell J,Lindquist E,Sabat G,Bondurant SS,Larrondo LF,Canessa P,Vic

更新日期：2009-02-10 00:00:00

abstract：:Break-induced replication (BIR) refers to recombination-dependent DNA synthesis initiated from one end of a DNA double-strand break and can extend for more than 100 kb. BIR initiates by Rad51-catalyzed strand invasion, but the mechanism for DNA synthesis is not known. Here, we used BrdU incorporation to track DNA synt...

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

authors： Donnianni RA,Symington LS

更新日期：2013-08-13 00:00:00

abstract：:Methylation on lysine 9 of histone H3 (H3K9me) and DNA methylation play important roles in the transcriptional silencing of specific genes and repetitive elements. Both marks are detected on class I and II endogenous retroviruses (ERVs) in murine embryonic stem cells (mESCs). Recently, we reported that the H3K9-specif...

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

authors： Leung DC,Dong KB,Maksakova IA,Goyal P,Appanah R,Lee S,Tachibana M,Shinkai Y,Lehnertz B,Mager DL,Rossi F,Lorincz MC

更新日期：2011-04-05 00:00:00

abstract：:Although gamma/delta T cells express numerous in vitro functions similar to alpha/beta T cells, little is known about their biological functioning in vivo. Furthermore, it is unclear whether alpha/beta T cells and gamma/delta T cells act independently or in a coordinated way. In the present study, gamma/delta T cells ...

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

authors： Kaufmann SH,Blum C,Yamamoto S

更新日期：1993-10-15 00:00:00

abstract：:Human papillomavirus type 18 (HPV-18) infects genital squamous epithelium and is an etiological agent of cervical cancer. Cell-type-specific expression of HPV-18 is directed by the region upstream of the viral early genes that contains a transcriptional enhancer whose function is dependent solely on cellular factors. ...

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

authors： Mack DH,Laimins LA

更新日期：1991-10-15 00:00:00

abstract：:Lewy bodies and Lewy neurites are the defining neuropathological characteristics of Parkinson's disease and dementia with Lewy bodies. They are made of abnormal filamentous assemblies of unknown composition. We show here that Lewy bodies and Lewy neurites from Parkinson's disease and dementia with Lewy bodies are stai...

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

authors： Spillantini MG,Crowther RA,Jakes R,Hasegawa M,Goedert M

更新日期：1998-05-26 00:00:00

abstract：:Many materials phenomena are governed by the interaction between chemistry and mechanics. However, it was only in the second half of the 20th century that the theory of open system elasticity by Francis Larché and John W. Cahn concatenated the fields of solid mechanics and alloy chemistry. As the theory's central mate...

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

authors： Shi S,Markmann J,Weissmüller J

更新日期：2018-10-23 00:00:00

abstract：:A 23-kilobase-pair segment of DNA containing the entire mouse serum albumin gene as well as 2.2 kilobase pairs of 5' and 4.3 kilobase pairs of 3' flanking sequences has been introduced into pSV2dhfr, a plasmid in which expression of the mouse dihydrofolate reductase cDNA is under the control of simian virus 40 sequenc...

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

authors： Deschatrette J,Fougere-Deschatrette C,Corcos L,Schimke RT

更新日期：1985-02-01 00:00:00

abstract：:The twin-arginine transport (Tat) system is a protein-targeting pathway of prokaryotes and chloroplasts. Most Escherichia coli Tat substrates are complex metalloenzymes that must be correctly folded and assembled before transport, and a preexport chaperone-mediated "proofreading" process is therefore in operation. The...

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

authors： Hatzixanthis K,Clarke TA,Oubrie A,Richardson DJ,Turner RJ,Sargent F

更新日期：2005-06-14 00:00:00

abstract：:The covalent attachment of heme to apocytochrome c, and therefore the import of cytochrome c into mitochondria, is dependent on both NADH plus a cytosolic cofactor that has been identified to be FMN or FAD. NADH in concert with flavin nucleotides mediates the reduction of heme. Heme in the reduced state is a prerequis...

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

authors： Nicholson DW,Neupert W

更新日期：1989-06-01 00:00:00

abstract：:To specify when and where Ig class switch recombination (CSR) takes place, a good molecular marker closely associated with active CSR is required. CSR is accompanied by deletion of circular DNA from the Ig heavy chain locus. The circular DNA contains a DNA segment between Smu and a target S region including its I prom...

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

authors： Kinoshita K,Harigai M,Fagarasan S,Muramatsu M,Honjo T

更新日期：2001-10-23 00:00:00

abstract：:X-linked hypophosphatemia is the most prevalent inherited form of rickets. In this disorder, rickets results from hyperphosphaturia and inappropriately normal levels of 1,25(OH)2-vitamin D. Current therapy with oral phosphate and vitamin D improves the rickets, but has significant morbidity and does not significantly ...

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

authors： Baum M,Loleh S,Saini N,Seikaly M,Dwarakanath V,Quigley R

更新日期：2003-09-16 00:00:00

abstract：:A promoterless trp-lac fusion fragment was inserted near one end of the bacterial transposon Tn5 in the correct orientation to fuse lacZ gene expression to promoters outside Tn5. The resulting transposon, Tn5 lac, retains the kanamycin-resistance gene of Tn5 and transposes in Escherichia coli at 6% the frequency of Tn...

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

authors： Kroos L,Kaiser D

更新日期：1984-09-01 00:00:00

abstract：:Although the applicability of small subunit ribosomal RNA (16S rRNA) sequences for bacterial classification is now well accepted, the general use of these molecules has been hindered by the technical difficulty of obtaining their sequences. A protocol is described for rapidly generating large blocks of 16S rRNA sequen...

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

authors： Lane DJ,Pace B,Olsen GJ,Stahl DA,Sogin ML,Pace NR

更新日期：1985-10-01 00:00:00

abstract：:The rapidly expanding network of roads into the Amazon is permanently altering the world's largest tropical forest. Most proposed road projects lack rigorous impact assessments or even basic economic justification. This study analyzes the expected environmental, social and economic impacts of 75 road projects, totalin...

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

authors： Vilela T,Malky Harb A,Bruner A,Laísa da Silva Arruda V,Ribeiro V,Auxiliadora Costa Alencar A,Julissa Escobedo Grandez A,Rojas A,Laina A,Botero R

更新日期：2020-03-31 00:00:00

abstract：:In the vertebrate central nervous system, the retina has been a useful model for studies of cell fate determination. Recent results from studies conducted in vitro and in vivo suggest a model of retinal development in which both the progenitor cells and the environment change over time. The model is based upon the not...

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

authors： Cepko CL,Austin CP,Yang X,Alexiades M,Ezzeddine D

更新日期：1996-01-23 00:00:00

abstract：:Messenger RNA from rat liver was partially purified by chromatography on cellulose on the basis of its poly(A) content. Microgram amounts of this RNA stimulate protein synthesis manyfold in a heterologous cell-free system, derived from Krebs ascites cells supplemented with reticulocyte initiation factors. The messenge...

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

authors： Schutz G,Beato M,Feigelson P

更新日期：1973-04-01 00:00:00

abstract：:The role of spliced leader RNA (SL RNA) in trans-splicing in Caenorhabditis elegans has been studied through a combination of in vitro mutagenesis and in vivo complementation of rrs-1 mutant nematodes, which lack endogenous SL1 RNA. Three classes of mutant SL1 RNAs have been found-those that rescue the lethal phenotyp...

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

authors： Xie H,Hirsh D

更新日期：1998-04-14 00:00:00

abstract：:The cytokines interleukin (IL) 4 and IL-13 induce many of the same biological responses, including class switching to IgE and induction of major histocompatibility complex class II antigens and CD23 on human B cells. It has recently been shown that IL-4 induces the tyrosine phosphorylation of a 170-kDa protein, a subs...

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

authors： Keegan AD,Johnston JA,Tortolani PJ,McReynolds LJ,Kinzer C,O'Shea JJ,Paul WE

更新日期：1995-08-15 00:00:00

abstract：:Diffusion in the plasma membrane of living cells is often found to display anomalous dynamics. However, the mechanism underlying this diffusion pattern remains highly controversial. Here, we study the physical mechanism underlying Kv2.1 potassium channel anomalous dynamics using single-molecule tracking. Our analysis ...

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

authors： Weigel AV,Simon B,Tamkun MM,Krapf D

更新日期：2011-04-19 00:00:00

abstract：:The Dbf4Cdc7 kinase acts at the level of individual origins to promote the initiation of DNA replication. We demonstrate through both immunoprecipitation and two-hybrid assays that a domain comprising the first 296 aa of Dbf4p interacts with Orc2p and Orc3p subunits of the origin recognition complex (ORC). Given that ...

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

authors： Duncker BP,Shimada K,Tsai-Pflugfelder M,Pasero P,Gasser SM

更新日期：2002-12-10 00:00:00

abstract：:Gametocytes are nonreplicative sexual forms that mediate malaria transmission to a mosquito vector. They are generated from asexual blood-stage parasites that proliferate in the circulation. However, little is known about how this transition is genetically regulated. Here, we report that an Apetala2 (AP2) family trans...

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

authors： Yuda M,Iwanaga S,Kaneko I,Kato T

更新日期：2015-10-13 00:00:00

abstract：:We used electron cryotomography and subtomogram averaging to determine the in situ structures of mitochondrial ATP synthase dimers from two organisms belonging to the phylum euglenozoa: Trypanosoma brucei, a lethal human parasite, and Euglena gracilis, a photosynthetic protist. At a resolution of 32.5 Å and 27.5 Å, re...

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

authors： Mühleip AW,Dewar CE,Schnaufer A,Kühlbrandt W,Davies KM

更新日期：2017-01-31 00:00:00