Severe infectious diseases of childhood as monogenic inborn errors of immunity.

abstract：:Livers of a natural population of winter flounder from a contaminated site in Boston Harbor were examined for the presence of oncogenes by transfection of DNA into NIH 3T3 mouse fibroblasts. Tissues analyzed contained histopathologic lesions including abnormal vacuolation, biliary proliferation, and, in many cases, he...

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

authors： McMahon G,Huber LJ,Moore MJ,Stegeman JJ,Wogan GN

更新日期：1990-01-01 00:00:00

abstract：:If they are able to spread in wild populations, CRISPR-based gene-drive elements would provide new ways to address ecological problems by altering the traits of wild organisms, but the potential for uncontrolled spread tremendously complicates ethical development and use. Here, we detail a self-exhausting form of CRIS...

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

authors： Noble C,Min J,Olejarz J,Buchthal J,Chavez A,Smidler AL,DeBenedictis EA,Church GM,Nowak MA,Esvelt KM

更新日期：2019-04-23 00:00:00

abstract：:The human immunodeficiency virus 1 (HIV-1) Tat protein suppresses antigen-induced, but not mitogen-induced, activation of human T cells when added to T-cell cultures [Viscidi, R. P., Mayur, K., Lederman, H. M. & Frankel, A. D. (1989) Science 246, 1606-1608]. This activity is potentially pertinent to the development of...

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

authors： Gutheil WG,Subramanyam M,Flentke GR,Sanford DG,Munoz E,Huber BT,Bachovchin WW

更新日期：1994-07-05 00:00:00

abstract：:The proliferating cell nuclear antigen (PCNA) acts as a processivity factor for replicative DNA polymerases and is essential for DNA replication. In vitro studies have suggested a role for PCNA-in the repair synthesis step of nucleotide excision repair, and PCNA interacts with the cyclin-dependent kinase inhibitor p21...

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

authors： Torres-Ramos CA,Yoder BL,Burgers PM,Prakash S,Prakash L

更新日期：1996-09-03 00:00:00

abstract：:SecA, the translocation ATPase in Escherichia coli, undergoes cycles of conformational changes (insertion/deinsertion) in response to ATP and a preprotein. The membrane-embedded portion of protein translocase, SecYEG, has crucial roles in the SecA-driven preprotein translocation reaction. We previously identified a se...

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

authors： Matsumoto G,Mori H,Ito K

更新日期：1998-11-10 00:00:00

abstract：:We have isolated a cold-sensitive mutant of Saccharomyces cerevisiae in which there is a deficit of 60S ribosomal subunits. Cold sensitivity and the assembly defect are recessive and cosegregate, defining a single essential gene that we designated DRS1 (deficiency of ribosomal subunits). The wild-type DRS1 gene was cl...

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

authors： Ripmaster TL,Vaughn GP,Woolford JL Jr

更新日期：1992-12-01 00:00:00

abstract：:Enzyme cytochemistry has been used, at the light and electron microscope levels, to "mark" cytoplasmic organelles of mammalian cells. Catalase cytochemistry permitted identification of microperoxisomes, apparently ubiquitous organelles that are attached by numerous slender connections to the endoplasmic reticulum. Thi...

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

authors： Novikoff AB

更新日期：1976-08-01 00:00:00

abstract：:Habitat loss and disease are main drivers of global amphibian declines, yet the interaction between them remains largely unexplored. Here we show that paradoxically, habitat loss is negatively associated with occurrence, prevalence, and infection intensity of the chytrid fungus Batrachochytrium dendrobatidis (Bd) in a...

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

authors： Becker CG,Zamudio KR

更新日期：2011-06-14 00:00:00

abstract：:As a direct simulation of a multistep proton transfer reaction involving protein residues, the proton relay shuttle between A and I forms of green fluorescent protein (GFP) is simulated in atomic detail by using a special molecular dynamics simulation technique. Electronic excitation of neutral chromophore in wild-typ...

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

authors： Lill MA,Helms V

更新日期：2002-03-05 00:00:00

abstract：:The Get3 ATPase directs the delivery of tail-anchored (TA) proteins to the endoplasmic reticulum (ER). TA-proteins are characterized by having a single transmembrane helix (TM) at their extreme C terminus and include many essential proteins, such as SNAREs, apoptosis factors, and protein translocation components. Thes...

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

authors： Suloway CJ,Chartron JW,Zaslaver M,Clemons WM Jr

更新日期：2009-09-01 00:00:00

abstract：:Experimental exclusion of the Andean flamingo (Phoenicoparrus andinus) from shallow water areas of a salt lake in the Bolivian Andes caused large increases in the biomass of microorganisms inhabiting the surface sediments, especially a large diatom (Surirella wetzeli), amebas, ciliates, and nematodes. This is a conser...

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

authors： Hurlbert SH,Chang CC

更新日期：1983-08-01 00:00:00

abstract：:Type VI protein secretion system (T6SS) is important for bacterial competition through contact-dependent killing of competitors. T6SS delivers effectors to neighboring cells and corresponding antagonistic proteins confer immunity against effectors that are delivered by sister cells. Although T6SS has been found in mor...

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

authors： Dong TG,Ho BT,Yoder-Himes DR,Mekalanos JJ

更新日期：2013-02-12 00:00:00

abstract：:The observation that the age-specific incidence curve of many carcinomas is approximately linear on a double logarithmic plot has led to much speculation regarding the number and nature of the critical events involved in carcinogenesis. By a consideration of colorectal and pancreatic cancers in the Surveillance Epidem...

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

authors： Meza R,Jeon J,Moolgavkar SH,Luebeck EG

更新日期：2008-10-21 00:00:00

abstract：:Cells have evolved complex regulatory networks that reorganize gene expression patterns in response to changing environmental conditions. These changes often involve redundant mechanisms that affect various levels of gene expression. Here, we examine the consequences of enhanced mRNA degradation in the galactose utili...

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

authors： Baumgartner BL,Bennett MR,Ferry M,Johnson TL,Tsimring LS,Hasty J

更新日期：2011-12-27 00:00:00

abstract：:The memory for a new episode is formed immediately upon experience and can last up to a lifetime. It has been shown that the hippocampal network plays a fundamental role in the rapid acquisition of a memory of a one-time experience, in which the novelty component of the experience promotes the prompt formation of the ...

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

authors： Wagatsuma A,Okuyama T,Sun C,Smith LM,Abe K,Tonegawa S

更新日期：2018-01-09 00:00:00

abstract：:The "false thumb" of pandas is a carpal bone, the radial sesamoid, which has been enlarged and functions as an opposable thumb. If the giant panda (Ailuropoda melanoleuca) and the red panda (Ailurus fulgens) are not closely related, their sharing of this adaptation implies a remarkable convergence. The discovery of pr...

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

authors： Salesa MJ,Antón M,Peigné S,Morales J

更新日期：2006-01-10 00:00:00

abstract：:Secondary (i.e., heterotrophic or animal) production is a main pathway of energy flow through an ecosystem as it makes energy available to consumers, including humans. Its estimation can play a valuable role in the examination of linkages between ecosystem functions and services. We found that oil and gas platforms of...

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

authors： Claisse JT,Pondella DJ 2nd,Love M,Zahn LA,Williams CM,Williams JP,Bull AS

更新日期：2014-10-28 00:00:00

abstract：:Membrane contact sites (MCSs) in eukaryotic cells are hotspots for lipid exchange, which is essential for many biological functions, including regulation of membrane properties and protein trafficking. Lipid transfer proteins anchored at membrane contact sites (LAMs) contain sterol-specific lipid transfer domains [StA...

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

authors： Tong J,Manik MK,Im YJ

更新日期：2018-01-30 00:00:00

abstract：:Sulfoquinovose (SQ; 6-deoxy-6-sulfoglucose) is the polar head group of the plant sulfolipid SQ-diacylglycerol, and SQ comprises a major proportion of the organosulfur in nature, where it is degraded by bacteria. A first degradation pathway for SQ has been demonstrated recently, a "sulfoglycolytic" pathway, in addition...

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

authors： Felux AK,Spiteller D,Klebensberger J,Schleheck D

更新日期：2015-08-04 00:00:00

abstract：:Mutations in fibroblast growth factor receptor 2 (FGFR2) and its ligand, FGF10, are known to cause lacrimo-auriculo-dento-digital (LADD) syndrome. Multiple gain-of-function mutations in FGF receptors have been implicated in a variety of severe skeletal disorders and in many cancers. We aimed to elucidate the mechanism...

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

authors： Lew ED,Bae JH,Rohmann E,Wollnik B,Schlessinger J

更新日期：2007-12-11 00:00:00

abstract：:"Stoppers" are a class of Neurospora crassa extranuclear mutants characterized by gross deficiencies of cytochromes b and aa3 and an unusual growth phenotype which involves irregular periods of growth andnongrowth. In the present work, mtDNAs from all four stopper mutants were found to contain deletions or insertions ...

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

authors： Bertrand H,Collins RA,Stohl LL,Goewert RR,Lambowitz AM

更新日期：1980-10-01 00:00:00

abstract：:In adipocytes, adenylate cyclase is positively regulated by beta-adrenergic agents and negatively regulated by adenosine. Incubation of adipocytes with adenosine deaminase relieves the inhibition of adenylate cyclase by destroying the adenosine that the cells release into the medium. When adipocytes are incubated with...

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

authors： Chung FZ,Weber HW,Appleman MM

更新日期：1985-03-01 00:00:00

abstract：:The apparent detection of an exoplanet orbiting Fomalhaut was announced in 2008. However, subsequent observations of Fomalhaut b raised questions about its status: Unlike other exoplanets, it is bright in the optical and nondetected in the infrared, and its orbit appears to cross the debris ring around the star withou...

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

authors： Gáspár A,Rieke GH

更新日期：2020-05-05 00:00:00

abstract：:Fishers with individual catch quota, but limited control over the mix of species caught, depend on trade and catch-quota balancing allowances to fully utilize their quota without discarding. However, these allowances can theoretically lead to overfishing if total allowable catches (TACs) are consistently exceeded. Thi...

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

authors： Oostdijk M,Byrne C,Stefánsson G,Santos MJ,Woods PJ

更新日期：2020-10-06 00:00:00

abstract：:Previous studies have defined transcriptional control elements, in addition to the promoters, that both lie near individual human beta-globin locus genes and have been implicated in their differential stage-specific regulation during development (i.e., are believed to directly participate in hemoglobin switching). We ...

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

authors： Liu Q,Bungert J,Engel JD

更新日期：1997-01-07 00:00:00

abstract：:Tight junctions (TJs) play a key role in mediating paracellular ion reabsorption in the kidney. The paracellular pathway in the collecting duct of the kidney is a predominant route for transepithelial chloride reabsorption that determines the extracellular NaCl content and the blood pressure. However, the molecular me...

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

authors： Hou J,Renigunta A,Yang J,Waldegger S

更新日期：2010-10-19 00:00:00

abstract：:Characterization and functional annotation of the large number of proteins predicted from genome sequencing projects poses a major scientific challenge. Whereas several proteomics techniques have been developed to quantify the abundance of proteins, these methods provide little information regarding protein function. ...

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

authors： Okerberg ES,Wu J,Zhang B,Samii B,Blackford K,Winn DT,Shreder KR,Burbaum JJ,Patricelli MP

更新日期：2005-04-05 00:00:00

abstract：:It is now well established that glasses feature quasilocalized nonphononic excitations-coined "soft spots"-, which follow a universal [Formula: see text] density of states in the limit of low frequencies ω. All glass-specific properties, such as the dependence on the preparation protocol or composition, are encapsulat...

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

authors： Rainone C,Bouchbinder E,Lerner E

更新日期：2020-03-10 00:00:00

abstract：:We have demonstrated the existence of human cytokine synthesis inhibitory factor (CSIF) [interleukin 10 (IL-10)]. cDNA clones encoding human IL-10 (hIL-10) were isolated from a tetanus toxin-specific human T-cell clone. Like mouse IL-10, hIL-10 exhibits strong DNA and amino acid sequence homology to an open reading fr...

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

authors： Vieira P,de Waal-Malefyt R,Dang MN,Johnson KE,Kastelein R,Fiorentino DF,deVries JE,Roncarolo MG,Mosmann TR,Moore KW

更新日期：1991-02-15 00:00:00

abstract：:Humans speak, monkeys grunt, and ducks quack. How do we come to know which vocalizations animals produce? Here we explore this question by asking whether young infants expect humans, but not other animals, to produce speech, and further, whether infants have similarly restricted expectations about the sources of vocal...

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

authors： Vouloumanos A,Druhen MJ,Hauser MD,Huizink AT

更新日期：2009-11-03 00:00:00