CNS expression of glucocerebrosidase corrects alpha-synuclein pathology and memory in a mouse model of Gaucher-related synucleinopathy.

abstract：:Using single-cell sequence analysis, we discovered that a high proportion of cells in tissues as diverse as buccal epithelium and heart muscle contain high proportions of clonal mutant mtDNA expanded from single initial mutant mtDNA molecules. We demonstrate that intracellular clonal expansion of somatic point mutatio...

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

authors： Nekhaeva E,Bodyak ND,Kraytsberg Y,McGrath SB,Van Orsouw NJ,Pluzhnikov A,Wei JY,Vijg J,Khrapko K

更新日期：2002-04-16 00:00:00

abstract：:We constructed a comprehensive cDNA library from HeLa cell mRNA in a vector that directs expression of the cDNA in Saccharomyces cerevisiae. We used this library to clone the human counterpart of the Sa. cerevisiae CCAAT-binding transcription factor, Hap2, by functional complementation of a hap2 mutation. The cDNA enc...

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

authors： Becker DM,Fikes JD,Guarente L

更新日期：1991-03-01 00:00:00

abstract：:Absolute pitch (AP) is the rare ability to identify the pitch of a tone without the aid of a reference tone. Understanding both the nature and genesis of AP can provide insights into neuroplasticity in the auditory system. We explored factors that may influence the accuracy of pitch perception in AP subjects both duri...

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

authors： Athos EA,Levinson B,Kistler A,Zemansky J,Bostrom A,Freimer N,Gitschier J

更新日期：2007-09-11 00:00:00

abstract：:USF is a family of transcription factors characterized by a highly conserved basic-helix-loop-helix-leucine zipper (bHLH-zip) DNA-binding domain. Two different USF genes, termed USF1 and USF2, are ubiquitously expressed in both humans and mice. The USF1 and USF2 proteins contain highly divergent transcriptional activa...

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

authors： Luo X,Sawadogo M

更新日期：1996-02-06 00:00:00

abstract：:Extensive regeneration of the vertebrate body plan is found in salamander and fish species. In these organisms, regeneration takes place through reprogramming of differentiated cells, proliferation, and subsequent redifferentiation of adult tissues. Such plasticity is rarely found in adult mammalian tissues, and this ...

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

authors： Yun MH,Gates PB,Brockes JP

更新日期：2013-10-22 00:00:00

abstract：:Monoclonal antibodies (mAbs) against purified human placental insulin-like growth factor I (IGF-I) receptors were prepared and characterized. Three IgG mAbs were specific for the human IGF-I receptor and displayed negligible crossreactivity with the human insulin receptor. They stimulated 125I-labeled IGF-I (125I-IGF-...

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

authors： Xiong L,Kasuya J,Li SL,Kato J,Fujita-Yamaguchi Y

更新日期：1992-06-15 00:00:00

abstract：:Mechanical forces have key roles in regulating activation of T cells and coordination of the adaptive immune response. A recent example is the ability of T cells to sense the rigidity of an underlying substrate through the T-cell receptor (TCR) coreceptor CD3 and CD28, a costimulation signal essential for cell activat...

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

authors： Bashour KT,Gondarenko A,Chen H,Shen K,Liu X,Huse M,Hone JC,Kam LC

更新日期：2014-02-11 00:00:00

abstract：:A 92-amino acid polypeptide, alpha-inhibin-92 (alpha-IB-92), has been isolated and characterized from human seminal plasma and found to be active in suppressing follicle-stimulating hormone (FSH) release in vitro. In the present in vivo study, intravenous injection of synthetic alpha-IB-92 (4 and 20 micrograms) signif...

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

authors： Yu WH,McCann SM,Li CH

更新日期：1988-01-01 00:00:00

abstract：:Replication of the single-stranded DNA genome of geminiviruses occurs via a double-stranded intermediate that is subsequently used as a template for rolling-circle replication of the viral strand. Only one of the proteins encoded by the virus, here referred to as replication initiator protein (Rep protein), is indispe...

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

authors： Laufs J,Traut W,Heyraud F,Matzeit V,Rogers SG,Schell J,Gronenborn B

更新日期：1995-04-25 00:00:00

abstract：:Tridecaptin A1 (TriA1) is a nonribosomal lipopeptide with selective antimicrobial activity against Gram-negative bacteria. Here we show that TriA1 exerts its bactericidal effect by binding to the bacterial cell-wall precursor lipid II on the inner membrane, disrupting the proton motive force. Biochemical and biophysic...

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

authors： Cochrane SA,Findlay B,Bakhtiary A,Acedo JZ,Rodriguez-Lopez EM,Mercier P,Vederas JC

更新日期：2016-10-11 00:00:00

abstract：:Prions are infectious proteins that encipher biological information within their conformations; variations in these conformations dictate different prion strains. Toward elucidating the molecular language of prion protein (PrP) conformations, we produced an array of recombinant PrP amyloids with varying conformational...

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

authors： Colby DW,Giles K,Legname G,Wille H,Baskakov IV,DeArmond SJ,Prusiner SB

更新日期：2009-12-01 00:00:00

abstract：:The SOS-inducible ruvA and ruvB gene products of Escherichia coli are required for normal levels of genetic recombination and DNA repair. In vitro, RuvA protein interacts specifically with Holliday junctions and, together with RuvB (an ATPase), promotes their movement along DNA. This process, known as branch migration...

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

authors： Tsaneva IR,Müller B,West SC

更新日期：1993-02-15 00:00:00

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

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

abstract：:Excitatory amino acid transporters (EAATs) buffer and remove synaptically released L-glutamate and maintain its concentrations below neurotoxic levels. EAATs also mediate a thermodynamically uncoupled substrate-gated anion conductance that may modulate cell excitability. Here, we demonstrate that modification of a cys...

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

authors： Seal RP,Shigeri Y,Eliasof S,Leighton BH,Amara SG

更新日期：2001-12-18 00:00:00

abstract：:Endothelial cells (ECs) line the mammalian vascular system and respond to the hemodynamic stimulus of fluid shear stress, the frictional force produced by blood flow. When ECs are exposed to shear stress, one of the fastest responses is an increase of K(+) conductance, which suggests that ion channels are involved in ...

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

authors： Hoger JH,Ilyin VI,Forsyth S,Hoger A

更新日期：2002-05-28 00:00:00

abstract：:Tubular membrane structures are widespread in eukaryotic cells, but the mechanisms underlying their formation and stability are not well understood. Previous work has focused on tube extrusion from cells and model membranes under the application of external forces. Here, we present novel membrane/polymer systems, wher...

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

authors： Li Y,Lipowsky R,Dimova R

更新日期：2011-03-22 00:00:00

abstract：:When expressed in Escherichia coli, the human beta 1- and beta 2-adrenergic receptors retain their ligand binding specificity. Their functional integrity was investigated by analyzing receptor-guanine nucleotide-binding regulatory (G) protein coupling by using two splice variants of the alpha subunit of the stimulator...

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

authors： Freissmuth M,Selzer E,Marullo S,Schütz W,Strosberg AD

更新日期：1991-10-01 00:00:00

abstract：:Metastasis, the truly lethal aspect of cancer, occurs when metastatic cancer cells in a tumor break through the basement membrane and penetrate the extracellular matrix. We show that MDA-MB-231 metastatic breast cancer cells cooperatively invade a 3D collagen matrix while following a glucose gradient. The invasion fro...

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

authors： Liu L,Duclos G,Sun B,Lee J,Wu A,Kam Y,Sontag ED,Stone HA,Sturm JC,Gatenby RA,Austin RH

更新日期：2013-01-29 00:00:00

abstract：:Mixing genomes of different species by hybridization can disrupt species-specific genetic interactions that were adapted and fixed within each species population. Such disruption can predispose the hybrids to abnormalities and disease that decrease the overall fitness of the hybrids and is therefore named as hybrid in...

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

authors： Lu Y,Sandoval A,Voss S,Lai Z,Kneitz S,Boswell W,Boswell M,Savage M,Walter C,Warren W,Schartl M,Walter R

更新日期：2020-11-24 00:00:00

abstract：:Diabetic peripheral neuropathy (DPN) is the most common complication in both type 1 and type 2 diabetes. Here we studied some phenotypic features of a well-established animal model of type 2 diabetes, the leptin receptor-deficient db(-)/db(-) mouse, and also the effect of long-term (6 mo) treatment with coenzyme Q10 (...

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

authors： Shi TJ,Zhang MD,Zeberg H,Nilsson J,Grünler J,Liu SX,Xiang Q,Persson J,Fried KJ,Catrina SB,Watanabe M,Arhem P,Brismar K,Hökfelt TG

更新日期：2013-01-08 00:00:00

abstract：:The structure of the proline-specific aminopeptidase (EC 3.4.11.9) from Escherichia coli has been solved and refined for crystals of the native enzyme at a 2.0-A resolution, for a dipeptide-inhibited complex at 2.3-A resolution, and for a low-pH inactive form at 2.7-A resolution. The protein crystallizes as a tetramer...

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

authors： Wilce MC,Bond CS,Dixon NE,Freeman HC,Guss JM,Lilley PE,Wilce JA

更新日期：1998-03-31 00:00:00

abstract：:By selective attachment of a DNA cleavage agent to specific residues in the yeast TATA box binding protein (yTBP), we demonstrate that, in solution, yTBP binds to the TATA boxes of both the adenovirus major late promoter and the yeast CYC1 promoter with only a modest preference in orientation and binds well to several...

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

authors： Cox JM,Hayward MM,Sanchez JF,Gegnas LD,van der Zee S,Dennis JH,Sigler PB,Schepartz A

更新日期：1997-12-09 00:00:00

abstract：:ATP-dependent DNA supercoiling catalyzed by Escherichia coli DNA gyrase was inhibited by oxolinic acid, a compound similar to but more potent than nalidixic acid and a known inhibitor of DNA replication in E. coli. The supercoiling activity of DNA gyrase purified from nalidixic acid-resistant mutant (nalA(R)) bacteria...

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

authors： Gellert M,Mizuuchi K,O'Dea MH,Itoh T,Tomizawa JI

更新日期：1977-11-01 00:00:00

abstract：:In a posterior silk gland extract, covalently closed circular (ccc) DNA is in a superhelical state that supports more transcription of fibroin gene than does linear DNA. A HeLa cell extract showed neither the supercoiling activity nor the preference for the transcription of ccc DNA over linear DNA. These activities co...

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

authors： Hirose S,Suzuki Y

更新日期：1988-02-01 00:00:00

abstract：:Many pathogenic bacteria produce extracellular DNase, but the benefit of this enzymatic activity is not understood. For example, all strains of the human bacterial pathogen group A Streptococcus (GAS) produce at least one extracellular DNase, and most strains make several distinct enzymes. Despite six decades of study...

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

authors： Sumby P,Barbian KD,Gardner DJ,Whitney AR,Welty DM,Long RD,Bailey JR,Parnell MJ,Hoe NP,Adams GG,Deleo FR,Musser JM

更新日期：2005-02-01 00:00:00

abstract：:The retrosplenial cortex (RSC) is part of a network of interconnected cortical, hippocampal, and thalamic structures harboring spatially modulated neurons. The RSC contains head direction cells and connects to the parahippocampal region and anterior thalamus. Manipulations of the RSC can affect spatial and contextual ...

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

authors： Czajkowski R,Jayaprakash B,Wiltgen B,Rogerson T,Guzman-Karlsson MC,Barth AL,Trachtenberg JT,Silva AJ

更新日期：2014-06-10 00:00:00

abstract：:The complete nucleotide sequences of the cDNAs encoding the heavy- and light-chain variable regions (VH and VL) of myeloma protein W3129, an anti-alpha(1----6)-dextran with a unique combining site, have been determined. The VH region is encoded by a germ-line gene highly homologous to VH441, which also appears to be u...

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

authors： Borden P,Kabat EA

更新日期：1987-04-01 00:00:00

abstract：:Lentiviruses, including HIV-1, have transmembrane envelope (Env) glycoproteins with cytoplasmic tails that are quite long compared with those of other retroviruses. However, mainly because of the lack of biochemical studies performed in cell types that are targets for HIV-1 infection, no clear consensus exists regardi...

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

authors： Murakami T,Freed EO

更新日期：2000-01-04 00:00:00

abstract：:Cytochrome c oxidase, the terminal protein in the respiratory chain, converts oxygen into water and helps generate the electrochemical gradient used in the synthesis of ATP. The catalytic action of cytochrome c oxidase involves electron transfer, proton transfer, and O2 reduction. These events trigger specific molecul...

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

authors： Nyquist RM,Heitbrink D,Bolwien C,Gennis RB,Heberle J

更新日期：2003-07-22 00:00:00

abstract：:We have previously shown that mammary epithelial specific expression of the activated erbB2 allele under the control of the endogenous promoter in mice resulted in the formation of mammary adenocarcinomas. To assess whether mammary tumorigenesis in this model is influenced by the developmental window of expression, we...

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

authors： Andrechek ER,Hardy WR,Laing MA,Muller WJ

更新日期：2004-04-06 00:00:00