Pelizaeus-Merzbacher disease: a valine to phenylalanine point mutation in a putative extracellular loop of myelin proteolipid.

abstract：:Horizontal drilling and hydraulic fracturing have enhanced energy production but raised concerns about drinking-water contamination and other environmental impacts. Identifying the sources and mechanisms of contamination can help improve the environmental and economic sustainability of shale-gas extraction. We analyze...

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

authors： Darrah TH,Vengosh A,Jackson RB,Warner NR,Poreda RJ

更新日期：2014-09-30 00:00:00

abstract：:Activation of G protein-coupled receptors (GPCRs) is triggered and regulated by structural rearrangement of the transmembrane heptahelical bundle containing a number of highly conserved residues. In rhodopsin, a prototypical GPCR, the helical bundle accommodates an intrinsic inverse-agonist 11-cis-retinal, which under...

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

authors： Okada T,Fujiyoshi Y,Silow M,Navarro J,Landau EM,Shichida Y

更新日期：2002-04-30 00:00:00

abstract：:Photochemical oxidation of aromatic hydrocarbons leads to tropospheric ozone and secondary organic aerosol (SOA) formation, with profound implications for air quality, human health, and climate. Toluene is the most abundant aromatic compound under urban environments, but its detailed chemical oxidation mechanism remai...

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

authors： Ji Y,Zhao J,Terazono H,Misawa K,Levitt NP,Li Y,Lin Y,Peng J,Wang Y,Duan L,Pan B,Zhang F,Feng X,An T,Marrero-Ortiz W,Secrest J,Zhang AL,Shibuya K,Molina MJ,Zhang R

更新日期：2017-08-01 00:00:00

abstract：:Kinesin is the canonical plus-end microtubule motor and has been the focus of intense study since its discovery in 1985. We previously demonstrated a time-dependent inactivation of kinesin in vitro that was fully reversible by the addition of purified casein kinase 2 (CK2) and showed that this inactivation/reactivatio...

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

authors： Mattson-Hoss MK,Niitani Y,Gordon EA,Jun Y,Bardwell L,Tomishige M,Gross SP

更新日期：2014-05-13 00:00:00

abstract：:Cholinergic pathways serve important functions in learning and memory processes, and deficits in cholinergic transmission occur in Alzheimer disease (AD). A subset of muscarinic cholinergic receptors are linked to G-proteins that activate phospholipase C, resulting in the liberation of inositol trisphosphate and Ca2+ ...

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

authors： Kelly JF,Furukawa K,Barger SW,Rengen MR,Mark RJ,Blanc EM,Roth GS,Mattson MP

更新日期：1996-06-25 00:00:00

abstract：:Plant metabolites are crucial for both plant life and human nutrition. Despite recent advance in metabolomics, the genetic control of plant metabolome remains largely unknown. Here, we performed a genetic analysis of the rice metabolome that provided over 2,800 highly resolved metabolic quantitative trait loci for 900...

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

authors： Gong L,Chen W,Gao Y,Liu X,Zhang H,Xu C,Yu S,Zhang Q,Luo J

更新日期：2013-12-10 00:00:00

abstract：:Gram-negative bacteria deliver a cadre of virulence factors directly into the cytoplasm of eukaryotic host cells to promote pathogenesis and/or commensalism. Recently, families of virulence proteins have been recognized that function as E3 Ubiquitin-ligases. How these bacterial ligases integrate into the ubiquitin (Ub...

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

authors： Levin I,Eakin C,Blanc MP,Klevit RE,Miller SI,Brzovic PS

更新日期：2010-02-16 00:00:00

abstract：:Leukocyte adherence to endothelium is in part mediated by the transient expression of endothelial-leukocyte adhesion molecule 1 (ELAM-1) on endothelial surfaces stimulated by tumor necrosis factor alpha (TNF), interleukin (IL) 1, or bacterial lipopolysaccharide (LPS). The intracellular factors controlling induction of...

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

authors： Montgomery KF,Osborn L,Hession C,Tizard R,Goff D,Vassallo C,Tarr PI,Bomsztyk K,Lobb R,Harlan JM

更新日期：1991-08-01 00:00:00

abstract：:The proteolytic processing of human amyloid precursor protein (APP) into shorter aggregating amyloid β (Aβ)-peptides, e.g., Aβ1-42, is considered a critical step in the pathogenesis of Alzheimer's disease (AD). Although APP is a well-known membrane glycoprotein carrying both N- and O-glycans, nothing is known about th...

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

authors： Halim A,Brinkmalm G,Rüetschi U,Westman-Brinkmalm A,Portelius E,Zetterberg H,Blennow K,Larson G,Nilsson J

更新日期：2011-07-19 00:00:00

abstract：:Echoviruses are enteroviruses that belong to Picornaviridae. Many echoviruses use decay-accelerating factor (DAF) as their cellular receptor. DAF is a glycosylphosphatidyl inositol-anchored complement regulatory protein found on most cell surfaces. It functions to protect cells from complement attack. The cryo-electro...

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

authors： He Y,Lin F,Chipman PR,Bator CM,Baker TS,Shoham M,Kuhn RJ,Medof ME,Rossmann MG

更新日期：2002-08-06 00:00:00

abstract：:The rate of derepressed synthesis of a membrane protein required for lactose transport (M protein) by Escherichia coli is increased in response to increased gene dosage to the same extent as the rates of synthesis of beta-galactosidase and galactoside acetylase. However, elevated gene dosage does not increase beta-gal...

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

authors： Fox CF

更新日期：1969-07-01 00:00:00

abstract：:High-resolution examination of pulse sequences generated by single visual cortex cells of the rhesus monkey in response to precisely controlled visual stimuli has disclosed (i) that the outputs of such neurons contain highly improbable (P less than 10(-7) numbers of identical triplets of precisely repeating pulse patt...

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

authors： Strehler BL,Lestienne R

更新日期：1986-12-01 00:00:00

abstract：:Hepatitis A virus (HAV) infects ∼1.4 million people annually and, although there is a vaccine, there are no licensed therapeutic drugs. HAV is unusually stable (making disinfection problematic) and little is known of how it enters cells and releases its RNA. Here we report a potent HAV-specific monoclonal antibody, R1...

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

authors： Wang X,Zhu L,Dang M,Hu Z,Gao Q,Yuan S,Sun Y,Zhang B,Ren J,Kotecha A,Walter TS,Wang J,Fry EE,Stuart DI,Rao Z

更新日期：2017-01-24 00:00:00

abstract：:Wnt/β-catenin signaling controls development and adult tissue homeostasis by regulating cell proliferation and cell fate decisions. Wnt binding to its receptors Frizzled (FZD) and low-density lipoprotein-related 6 (LRP6) at the cell surface initiates a signaling cascade that leads to the transcription of Wnt target ge...

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

authors： Gerlach JP,Jordens I,Tauriello DVF,van 't Land-Kuper I,Bugter JM,Noordstra I,van der Kooij J,Low TY,Pimentel-Muiños FX,Xanthakis D,Fenderico N,Rabouille C,Heck AJR,Egan DA,Maurice MM

更新日期：2018-04-24 00:00:00

abstract：:An improved electron cloud bolometer is analyzed. In this device the cloud temperature is read out not via thermal noise induced by the electrons in a coupled LC circuit but via shift in their axial oscillation frequency in the Penning trap confining them. This shift occurs because as the electron cloud expands with i...

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

authors： Dehmelt H

更新日期：1994-07-05 00:00:00

abstract：:Hereditary hemochromatosis (HH) is a common autosomal recessive disorder of iron metabolism that leads to excessive iron storage in the liver and other organs. Recently, between 83 and 100% of HH patients have been found to be homozygous for the same mutation in a novel major histocompatibility complex class I-like ge...

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

authors： Parkkila S,Waheed A,Britton RS,Feder JN,Tsuchihashi Z,Schatzman RC,Bacon BR,Sly WS

更新日期：1997-03-18 00:00:00

abstract：:We have previously described a gene delivery system based upon the receptor-mediated endocytosis of DNA complexed with transferrin-polycation conjugates. This delivery system has been found to be very effective for both the internalization and the expression of genetic material in cells that have many transferrin rece...

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

authors： Wagner E,Cotten M,Foisner R,Birnstiel ML

更新日期：1991-05-15 00:00:00

abstract：:We demonstrate how a conventional confocal spinning-disk (CSD) microscope can be converted into a doubly resolving image scanning microscopy (ISM) system without changing any part of its optical or mechanical elements. Making use of the intrinsic properties of a CSD microscope, we illuminate stroboscopically, generati...

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

authors： Schulz O,Pieper C,Clever M,Pfaff J,Ruhlandt A,Kehlenbach RH,Wouters FS,Großhans J,Bunt G,Enderlein J

更新日期：2013-12-24 00:00:00

abstract：:The diabetogenic agent alloxan exerts a preferential cytotoxic effect on the pancreatic B cell. The determinants of such a tissue specificity were investigated. Alloxan accumulated rapidly in liver and pancreatic islets but much more slowly in muscle. The activity of glutathione peroxidase and the resistance to exogen...

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

authors： Malaisse WJ,Malaisse-Lagae F,Sener A,Pipeleers DG

更新日期：1982-02-01 00:00:00

abstract：:1,N(6)-Ethenoadenosine triphosphate (epsilon-ATP) has been found to be inactive as a substrate for firefly luciferase. However, chemically synthesized luciferyl-epsilonAMP is oxidized by luciferase and light is emitted. The color of the light is red in contrast to the normal yellow-green observed with luciferyladenyla...

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

authors： Deluca M,Leonard NJ,Gates BJ,McElroy WD

更新日期：1973-06-01 00:00:00

abstract：:Controls of stem cell maintenance and early differentiation are known in several systems. However, the progression from stem cell self-renewal to overt signs of early differentiation is a poorly understood but important problem in stem cell biology. The Caenorhabditis elegans germ line provides a genetically defined m...

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

authors： Cinquin O,Crittenden SL,Morgan DE,Kimble J

更新日期：2010-02-02 00:00:00

abstract：:Transgenic mice have provided invaluable information about gene function and regulation. However, because of marked differences between rodents and primates, some areas of human biology such as early embryonic development, aging, and maternal-fetal interactions would be best studied in a nonhuman primate model. Here, ...

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

authors： Wolfgang MJ,Eisele SG,Browne MA,Schotzko ML,Garthwaite MA,Durning M,Ramezani A,Hawley RG,Thomson JA,Golos TG

更新日期：2001-09-11 00:00:00

abstract：:Cystic fibrosis (CF) is an autosomal recessive disorder, the most common lethal genetic disease in Caucasians. Respiratory disease is the major cause of morbidity and mortality. Indeed, 95% of CF patients die of respiratory failure. Pseudomonas aeruginosa, an opportunistic pathogen, chronically infects the lungs of ov...

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

authors： Li JD,Feng W,Gallup M,Kim JH,Gum J,Kim Y,Basbaum C

更新日期：1998-05-12 00:00:00

abstract：:Fuchs endothelial corneal dystrophy (FECD) is a leading cause of corneal endothelial (CE) degeneration resulting in impaired visual acuity. It is a genetically complex and age-related disorder, with higher incidence in females. In this study, we established a nongenetic FECD animal model based on the physiologic outco...

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

authors： Liu C,Miyajima T,Melangath G,Miyai T,Vasanth S,Deshpande N,Kumar V,Ong Tone S,Gupta R,Zhu S,Vojnovic D,Chen Y,Rogan EG,Mondal B,Zahid M,Jurkunas UV

更新日期：2020-01-07 00:00:00

abstract：:The mechanism by which cells recognize and complete replicated regions at their precise doubling point must be remarkably efficient, occurring thousands of times per cell division along the chromosomes of humans. However, this process remains poorly understood. Here we show that, in Escherichia coli, the completion of...

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

authors： Wendel BM,Courcelle CT,Courcelle J

更新日期：2014-11-18 00:00:00

abstract：:In this study, we demonstrate that fluid shear stress (FSS)-induced actin cytoskeletal reorganization and junctional formation in renal epithelial cells are nearly completely opposite the corresponding changes in vascular endothelial cells (ECs) [Thi MM et al. (2004) Proc Natl Acad Sci USA 101:16483-16488]. Mouse prox...

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

authors： Duan Y,Gotoh N,Yan Q,Du Z,Weinstein AM,Wang T,Weinbaum S

更新日期：2008-08-12 00:00:00

abstract：:The nanoscale distributions of electron density and electric fields in GaAs semiconductor devices are displayed with NMR experiments. The spectra are sensitive to the changes to the nuclear-spin Hamiltonian that are induced by perturbations delivered in synchrony with a line-narrowing pulse sequence. This POWER (pertu...

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

authors： Kempf JG,Miller MA,Weitekamp DP

更新日期：2008-12-23 00:00:00

abstract：:It is well-established morphologically that bipolar cells, the second-order neurons in the vertebrate retina, make reciprocal synapses with amacrine cells in the inner plexiform layer. However, neither the property nor the physiological function of the feedback synapse is understood. Autoradiographic and immunohistoch...

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

authors： Tachibana M,Kaneko A

更新日期：1987-05-01 00:00:00

abstract：:An unusual system of genetic exchange exists in the purple nonsulfur bacterium Rhodobacter capsulatus. DNA transmission is mediated by a small bacteriophage-like particle called the gene transfer agent (GTA) that transfers random 4.5-kb segments of the producing cell's genome to recipient cells, where allelic replacem...

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

authors： Lang AS,Beatty JT

更新日期：2000-01-18 00:00:00

abstract：:Animals treated with potent gamma-glutamyl transpeptidase inhibitors and a patient with severe gamma-glutamyl transpeptidase deficiency excrete much larger than normal amounts of glutathione, gamma-glutamylcysteine, and cysteine in their urine; these compounds were found in disulfide forms. The findings indicate that ...

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

authors： Griffith OW,Meister A

更新日期：1980-06-01 00:00:00