bluuebunny/crossref_metadata_2025_split huggingface dataset:
Myeloid dysplasia and bone marrow hypocellularity in adenosine deaminase-deficient severe combined immune deficiency
Authors: Sokolic Robert , Maric Irina , Kesserwan Chimene , Garabedian Elizabeth , Hanson I. Celine , Dodds Margaret , Buckley Rebecca , Issekutz Andrew C. , Kamani Naynesh , Shaw Kit , Tan Ben , Bali Pawan , Hershfield Michael S. , Kohn Donald B. , Wayne Alan S. , Candotti Fabio 
Published: July 2011

AbstractGenetic deficiency of adenosine deaminase (ADA) can cause profound lymphopenia and result in the clinical presentation of severe combined immune deficiency (SCID). However, because of the ubiquitous expression of ADA, ADA-deficient patients often present also with nonimmunologic clinical problems, affecting the skeletal, central nervous, endocrine, and gastrointestinal systems. We now report that myeloid dysplasia features and bone marrow hypocellularity are often found in patients with ADA-SCID. As a clinical correlate to this finding, we have observed vulnerability to antibiotic-induced myelotoxicity and prolonged neutropenia after nonmyeloablative chemotherapy. We have also noted that, in the absence of enzyme replacement therapy, absolute neutrophil counts of patients with ADA deficiency vary inversely with the accumulation of deoxynucleotides. These data have significant implications for the application of standard and investigational therapies to patients with ADA-SCID and support further studies to investigate the possibility that ADA deficiency is associated with a stem cell defect. These trials were registered at www.clinicaltrials.gov as #NCT00018018 and #NCT00006319.
Safety and efficacy of everolimus, a mTOR inhibitor, as single agent in a phase 1/2 study in patients with myelofibrosis
Authors: Guglielmelli Paola , Barosi Giovanni , Rambaldi Alessandro , Marchioli Roberto , Masciulli Arianna , Tozzi Lorenzo , Biamonte Flavia , Bartalucci Niccolò , Gattoni Elisabetta , Lupo Maria Letizia , Finazzi Guido , Pancrazzi Alessandro , Antonioli Elisabetta , Susini Maria Chiara , Pieri Lisa , Malevolti Elisa , Usala Emilio , Occhini Ubaldo , Grossi Alberto , Caglio Silvia , Paratore Simona , Bosi Alberto , Barbui Tiziano , Vannucchi Alessandro M. ,   on behalf of the AIRC-Gruppo Italiano Malattie Mieloproliferative (AGIMM) investigators
Published: July 2011

AbstractIn addition to dysregulated JAK/STAT signaling, activation of the AKT/mTOR pathway occurs in myelofibrosis, a myeloproliferative neoplasm with no approved therapies. We conducted a phase 1/2 study with everolimus, an mTOR inhibitor, in 39 high- or intermediate-risk primary or postpolycythemia vera/postessential thrombocythemia myelofibrosis subjects. Responses were evaluated in 30 patients of phase 2. No dose-limiting toxicity was observed in phase 1 up to 10 mg/d. When this dose was used in phase 2, grade ≥ 3 toxicities were infrequent; the commonest toxicity was grade 1-2 stomatitis. Rapid and sustained splenomegaly reduction of > 50% and > 30% occurred in 20% and 44% of subjects, respectively. A total of 69% and 80% experienced complete resolution of systemic symptoms and pruritus. Response in leukocytosis, anemia, and thrombocytosis occurred in 15%-25%. Clinical responses were not associated with reduced JAK2V617F burden, circulating CD34+ cells, or cytokine levels, whereas CCDN1 mRNA and phospho-p70S6K level, known targets of mTOR, and WT1 mRNA were identified as possible biomarkers associated with response. Response rate was 60% when European Network for Myelofibrosis criteria were used (8 major, 7 moderate, 3 minor responses) or 23% when IWG-MRT criteria (1 partial response, 6 clinical improvements) were used. These results provide proof-of-concept that targeting mTOR pathway in myelofibrosis may be clinically relevant.
Characterizing Hydrogen Bonding in Uracil–Nitrosamine Complexes through One- and Two-Bond Spin–Spin Coupling Constants across Hydrogen Bonds
Authors: Roohi Hossein , Anjomshoa Elham 
Published: July 2011

Abstract
One- and two-bond spin–spin coupling constants, 1J, 1hJ, and 2hJ across X–H···Y hydrogen bonds and isotropic chemical shift of bridging hydrogens have been computed for complexes formed from interaction between the parent nitrosamine (NA) and four preferential binding sites of the uracil (U) at B3LYP/6-311++G(2d,2p)//MP2/6-311++G(2d,2p) level of theory. All complexes are stabilized by two HU···Y and HNA···Y hydrogen bonds. The correlation between spin–spin coupling constants (1hJH···Y and 2hJX···Y) and isotropic chemical shifts with the binding energy, H-bond distance, red shift of vibration frequency, charge-transfer energy, and electron density at H-bond critical point were investigated at the above-mentioned level of theory. Change in nucleus-independent chemical shift of U ring upon complex formation was also calculated.
Molecular Optical Switch Based on Polymer Nanosheet Assembly Operated at Visible Wavelength
Authors: Matsui Jun , Abe Kenichi , Mitsuishi Masaya , Aoki Atsushi , Miyashita Tokuji 
Published: July 2011

Abstract
We constructed a molecular optical switch, which controls the photocurrent direction using a polymer nanosheet assembly. Two polymer nanosheet photodiodes composed of ruthenium complex (sensitizer) and ferrocene (donor) polymer nanosheet pair and anthracene (sensitizer) and dinitrobenzene (acceptor) polymer nanosheet pair, respectively, were assembled on the ITO electrode. Selective excitation of ruthenium by 450 nm generated cathodic photocurrent, whereas excitation of anthracene at 389 nm generated anodic photocurrent. The results indicate that the current flow direction in the assembled film can be controlled by the irradiation wavelength.
The role of CRF family peptides in the regulation of food intake and anxiety-like behavior
Authors: Nakayama Naoko , Suzuki Hajime , Li Jiang-Bo , Atsuchi Kaori , Tsai Minglun , Amitani Haruka , Asakawa Akihiro , Inui Akio 
Published: July 2011

AbstractCorticotropin-releasing factor (CRF) and the urocortins (UCN1, UCN2, and UCN3) belong to the CRF family of peptides and are the major regulators of the adaptive response to internal and external stresses. The actions of CRF and UCNs are mediated through two receptor subtypes: CRF receptor 1 (CRFR1) and CRFR2. Their physiological roles, among other functions, include the regulation of food intake and anxiety-like behavior. In this review, we describe the progress that has been made towards understanding how anxiety- and depression-like behavior and food intake are regulated by CRF, UCN1, UCN2, and UCN3.
The function and architecture of DEAH/RHA helicases
Authors: He Yangzi , Andersen Gregers R. , Nielsen Klaus H. 
Published: July 2011

AbstractHelicases are ubiquitous enzymes that participate in every aspect of nucleic acid metabolism. The DEAH/RHA family of helicases are involved in a variety of cellular processes including transcriptional and translational regulation, pre-mRNA splicing, pre-rRNA processing, mRNA export and decay, in addition to the innate immune response. Recently, the first crystal structures of a DEAH/RHA helicase unveiled the unique structural features of this helicase family. These structures furthermore illuminate the molecular mechanism of these proteins and provide a framework for analysis of their interaction with nucleic acids, regulatory proteins and large macromolecular complexes.
New oscillation criteria for third order nonlinear neutral difference equations
Authors: Saker S. 
Published: July 2011

Abstract
In this paper, we are concerned with oscillation of the third-order nonlinear neutral difference equation $\Delta (c_n [\Delta (d_n \Delta (x_n + p_n x_{n - \tau } ))]^\gamma ) + q_n f(x_{g(n)} ) = 0,n \geqslant n_0 ,$ where γ > 0 is the quotient of odd positive integers, c
n, d
n, p
n and q
n are positive sequences of real numbers, τ is a nonnegative integer, g(n) is a sequence of nonnegative integers and f ∈ C(ℝ,ℝ) such that uf(u) > 0 for u ≠ 0. Our results extend and improve some previously obtained ones. Some examples are considered to illustrate the main results.
Report of the Section of the Committee for Civil Engineering in the Term 2007–2010 / Sekcja Inzynierii Przedsiewziec Budowlanych W Kadencji 2007 – 2010. Raport
Authors: Kapliński O 
Published: July 2011

Abstract This report describes the community involved in technology, organisation, and economics of the construction industry. The community includes mainly academics gathered around KILiW PAN, the Polish Academy of Sciences Section for Civil Engineering Projects. The results have been obtained based on the survey conducted in the period of 2007-2010. Some financial issues are presented here which influence the scope of the research underway and the didactic process. Some of the issues presented here comprise the subject matters of research, postgraduate studies, academic degrees, as well as publications and international cooperation. Conclusions were presented in the form of suggestions in the field of research and teaching
Analysis of Soft Soil Consolidation with the Application of Prefabricated Vertical Drains with Preloading Method Using Fem / Analiza Konsolidacji Słabych Gruntów Przy Uzyciu Metody Elementów Skonczonych
Authors: Binder K. 
Published: July 2011

Abstract The article presents the application of finite element method for estimating settlements of road embankments founded on the soil reinforced with vertical drains and preloading method. The idea of the method was the transition from the solution of one-dimensional consolidation proposed for two-dimensional solution, while maintaining the same consolidation time and comparison with results obtained from measurements settlements of road embankment which is a part of planned Gdansk Southern Ring Road near Przejazdowo site.
Advanced Field Investigations of Screw Piles and Columns / Zaawansowane Badania Terenowe Pali I Kolumn Wkrecanych
Authors: Krasiński A. 
Published: July 2011

Abstract Field investigations concerning screw piles and columns have been carried out for the “Bearing capacity and work in the soil of screw piles” research project, financed by the Polish Ministry of Science and Higher Education - project No N N506 369234. The tests of three instrumented screw piles were conducted together with CPTU tests and measurements of pile installation parameters (especially torque). The objectives of field investigations and the entire research project include discovering how screw piles work in the soil, locating and describing the correlations between CPTU results and rotation resistance during pile auger installation and next establishing correlations between CPTU results, rotation resistance and the bearing capacity of this kind of piles. The paper describes the investigation procedure and the basic results of tests carried out in the first of a series of sites.
Baselines for the Preparation of Electronic Textbooks
Authors: Lokar Matija , Horvat Boris , Lukšič Primož , Omerza Damijan 
Published: July 2011

Baselines for the Preparation of Electronic Textbooks
The NAUK group (Advanced Learning Blocks group; http://www.nauk.si) is engaged in the development of theoretical and practical concepts of ICT use in all levels of education. Recently, the proposed introduction of electronic textbooks (e-textbooks) has become a major topic nationwide in Slovenia. There are multiple dilemmas to be solved, e.g. what a modern e-textbook is, what it should include, in what way should it differ from conventional textbooks, what tools and technologies should be used for its creation, etc. This paper attempts to make recommendations, which we believe should be followed by all the authors of e-textbooks.
The Evaluation of the Initial Skew Rate for Printed Text
Authors: Brodić Darko 
Published: July 2011

The Evaluation of the Initial Skew Rate for Printed TextIn this manuscript the algorithm for identification of the initial skew rate for printed text is presented. Proposed algorithm creates rectangular hull around all text characters. Combining nearby rectangular hulls form objects. After applying mathematical morphology on it, the biggest object is characterized as well as selected. Rectangular hull gravity center forms reference points on these objects used as a base for calculationieestimation of the initial skew rate. Using the least square method, initial skew rate is calculated. Comparative analysis of the origin and estimated skew rate is presented as well as discussed. Algorithm is examined with a number of printed text examples. Proposed algorithm showed robustness for skewness of printed text in the wide range.
New Evolutionary Technique for Optimization Shunt Capacitors in Distribution Networks
Authors: Elmaouhab Ali , Boudour Mohamed , Gueddouche Rabah 
Published: July 2011

New Evolutionary Technique for Optimization Shunt Capacitors in Distribution NetworksThe paper presents a new evolutionary technique for optimizing on one part the numbers, the sizes and locations of shunt capacitors in radial distribution network in away to minimize the annual cost of active power losses with the improvement of voltage profiles of different buses. The technique is applied on 10 buses network and on IEEE 34 buses network test. The results are compared with ones of previous studies using heuristiques methods and the same network tests.
The lex Valeria and Sulla’s empowerment as dictator (82-79 BCE)
Authors: Vervaet Frederik Juliaan 
Published: July 2011

In November 82 BCE, the victorious proconsul L. Cornelius Sulla (cos. 88) revived the dictatorship to restore law and order in the battered Republic. L. Valerius Flaccus (cos. 100), then
princeps senatus, was appointed interrex by the patrician patres. After obtaining the necessary clearances from the Senate and the augural college, Flaccus passed a law ordering him to appoint Sulla to an unprecedented dictatura legibus scribundis et rei publicae constituendae, and himself to the office of magister equitum, and this ad tempus incertum, until the completion of their vast commission. Although Sulla was thus invested with a series of spectacular extraordinary powers, he still scrupulously bothered to involve SPQR in the execution of his momentous political programme. Probably yet before the comitia consularia of 81, Sulla next initiated a gradual and carefully staged return to normalcy. The cornerstone of this policy was his decision to run the Republic in 80 as consul II, in conjunction with Q. Caecilius Metellus Pius. Its climax was Sulla’s dramatic abdication of the dictatorship at the outset of 79, proclaiming that he had now fully restored the Republic.
Les cités face à la damnatio memoriae : les martelages dans l’espace urbain
Authors: Lefebvre Sabine 
Published: July 2011

Dans le cadre de la constitution d’un corpus des documents martelés, notre réflexion s’est portée dans un premier temps sur l’identification du martelage et ses liens avec une éventuelle
damnatio memoriae. Il faut en effet éviter d’intégrer des documents mutilés par le temps et n’ayant rien à voir avec la condamnation de la mémoire, ou mal interprétés dans un premier temps, ou encore corrigés lors de leur mise en place en raison d’une erreur du lapicide, enfin, dégradés par les chrétiens. Le texte retenu étant clairement identifié comme ayant été mutilé à la suite d’une damnatio memoriae, il faut essayer de comprendre le contexte et les raisons de cette condamnation, privée ou publique; pour cela, nous disposons entre autres du sénatus-consulte contre Pison, qui nous fournit un grand nombre d’informations sur le martelage des documents concernant les hauts personnages de l’Empire et sur la diffusion des ordres émanant de Rome touchant la condamnation de la mémoire d’un personnage, mémoire qui reste néanmoins perceptible dans les espaces civiques, les bases de statues étant souvent maintenues en place.
Monnaie et damnatio memoriae (Ier-IVe siècle ap. J.-C.) : problèmes méthodologiques
Authors: Hostein Antony 
Published: July 2011

La présente contribution cherche à mesurer l’apport spécifique ainsi que les limites de la documentation numismatique pour étudier la procédure officielle de la damnatio memoriae,
depuis les Julio-Claudiens jusqu’au milieu du IIIe siècle. Un examen critique des principaux travaux publiés sur la question oblige à opérer un tri entre les monnaies injuriées dans le cadre d’une procédure officielle d’une part, et les autres d’autre part, et permet d’établir que les monnaies relevant de la première catégorie demeurent extrêmement rares. Ce constat s’explique pour diverses raisons (rôle économique de la monnaie; objet de petite taille se prêtant peu à des cérémonies exemplaires, etc.). Au final, il apparaît que la monnaie, comparée aux inscriptions et aux papyrus par exemple, constitue une documentation annexe et mineure pour la compréhension de cette procédure institutionnelle.
The Distribution of Congenital Anomalies Within the VACTERL Association Among Tumor Necrosis Factor Antagonist-exposed Pregnancies Is Similar to the General Population
Authors: CRIJNS HUBERTINA J.M.J. , JENTINK JANNEKE , GARNE ESTER , GISPEN-de WIED CHRISTINE C. , STRAUS SABINE M.J.M. , de JONG-van den BERG LOLKJE T.W. 
Published: July 2011

Objective.To compare the distribution of congenital anomalies within the VACTERL association (vertebral defects, anal atresia, cardiac, tracheoesophageal, renal, and limb abnormalities) between patients exposed to tumor necrosis factor-α (TNF-α) antagonist and the general population.Methods.Analysis for comparison of proportional differences to a previous publication between anomaly subgroups, according to subgroup definitions of the European Surveillance of Congenital Anomalies (EUROCAT), a population-based database.Results.Most EUROCAT subgroups belonging to the VACTERL association contained only one or 2 records of TNF-α antagonist exposure, so comparison of proportions was imprecise. Only the category “limb abnormalities” showed a significantly higher proportion in the general population.Conclusion.The high number of congenital anomalies belonging to the VACTERL association from a report of pregnancies exposed to TNF-α antagonists could not be confirmed using a population-based congenital anomaly database.
Innate Immune Collectin Surfactant Protein D Simultaneously Binds Both Neutrophil Extracellular Traps and Carbohydrate Ligands and Promotes Bacterial Trapping
Authors: Douda David Nobuhiro , Jackson Richard , Grasemann Hartmut , Palaniyar Nades 
Published: July 2011

Abstract
Neutrophils release DNA-based extracellular traps to capture and kill bacteria. The mechanism(s) and proteins that promote neutrophil extracellular trap (NET)-mediated bacterial trapping are not clearly established. Surfactant protein D (SP-D) is an innate immune collectin present in many mucosal surfaces. We hypothesized that SP-D can bind both the pathogens and NETs to augment NET-mediated bacterial trapping. To test this hypothesis, we used LPS and Pseudomonas aeruginosa pneumonia mouse models and performed in vivo and ex vivo assays. In this study, we show that NETs are produced by the neutrophils recruited to the airways in response to the bacterial ligand. Notably, NETs are detected as short fragments of DNA–protein complexes in the airways as opposed to the long stringlike structures seen in ex vivo cultures. SP-D recognizes both the short NET fragments and the long NET DNA structures. SP-D–NET copurification studies further show that SP-D can simultaneously recognize NETs and carbohydrate ligands in vivo. Similar to the LPS model, soluble DNA–protein complexes and increased amounts of SP-D are detected in the murine model of P. aeruginosa pneumonia. We then tested the effect of SP-D on NET-mediated trapping of P. aeruginosa by means of Western blots, fluorescence microscopy, and scanning electron microscopy. Results of these experiments show that SP-D microagglutinates P. aeruginosa and allows an efficient bacterial trapping by NETs. Collectively, these findings provide a unique biological relevance for SP-D–DNA interactions and places SP-D as an important innate immune protein that promotes bacterial trapping by NETs during neutrophil-mediated host defense.
Protective Roles for Fibrin, Tissue Factor, Plasminogen Activator Inhibitor-1, and Thrombin Activatable Fibrinolysis Inhibitor, but Not Factor XI, during Defense against the Gram-Negative BacteriumYersinia enterocolitica
Authors: Luo Deyan , Szaba Frank M. , Kummer Lawrence W. , Plow Edward F. , Mackman Nigel , Gailani David , Smiley Stephen T. 
Published: July 2011

AbstractSeptic infections dysregulate hemostatic pathways, prompting coagulopathy. Nevertheless, anticoagulant therapies typically fail to protect humans from septic pathology. The data reported in this work may help to explain this discrepancy by demonstrating critical protective roles for coagulation leading to fibrin deposition during host defense against the Gram-negative bacterium Yersinia enterocolitica. After i.p. inoculation with Y. enterocolitica, fibrinogen-deficient mice display impaired cytokine and chemokine production in the peritoneal cavity and suppressed neutrophil recruitment. Moreover, both gene-targeted fibrinogen-deficient mice and wild-type mice treated with the anticoagulant coumadin display increased hepatic bacterial burden and mortality following either i.p. or i.v. inoculation with Y. enterocolitica. Mice with low tissue factor activity succumb to yersiniosis with a phenotype similar to fibrin(ogen)-deficient mice, whereas factor XI-deficient mice show wild-type levels of resistance. Mice deficient in plasminogen activator inhibitor-1 or thrombin-activatable fibrinolysis inhibitor display modest phenotypes, but mice deficient in both plasminogen activator inhibitor-1 and thrombin-activatable fibrinolysis inhibitor succumb to yersiniosis with a phenotype resembling fibrin(ogen)-deficient mice. These findings demonstrate critical protective roles for the tissue factor-dependent extrinsic coagulation pathway during host defense against bacteria and caution that therapeutics targeting major thrombin-generating or antifibrinolytic pathways may disrupt fibrin-mediated host defense during Gram-negative sepsis.
The Inhibiting Fc Receptor for IgG, FcγRIIB, Is a Modifier of Autoimmune Susceptibility
Authors: Boross Peter , Arandhara Victoria L. , Martin-Ramirez Javier , Santiago-Raber Marie-Laure , Carlucci Francesco , Flierman Roelof , van der Kaa Jos , Breukel Cor , Claassens Jill W. C. , Camps Marcel , Lubberts Erik , Salvatori Daniela , Rastaldi Maria Pia , Ossendorp Ferry , Daha Mohamed R. , Cook H. Terence , Izui Shozo , Botto Marina , Verbeek J. Sjef 
Published: July 2011

Abstract
FcγRIIB-deficient mice generated in 129 background (FcγRIIB129−/−) if back-crossed into C57BL/6 background exhibit a hyperactive phenotype and develop lethal lupus. Both in mice and humans, the Fcγr2b gene is located within a genomic interval on chromosome 1 associated with lupus susceptibility. In mice, the 129-derived haplotype of this interval, named Sle16, causes loss of self-tolerance in the context of the B6 genome, hampering the analysis of the specific contribution of FcγRIIB deficiency to the development of lupus in FcγRIIB129−/− mice. Moreover, in humans genetic linkage studies revealed contradictory results regarding the association of “loss of function” mutations in the Fcγr2b gene and susceptibility to systemic lupus erythematosis. In this study, we demonstrate that FcγRIIB−/− mice generated by gene targeting in B6-derived ES cells (FcγRIIBB6−/−), lacking the 129-derived flanking Sle16 region, exhibit a hyperactive phenotype but fail to develop lupus indicating that in FcγRIIB129−/− mice, not FcγRIIB deficiency but epistatic interactions between the C57BL/6 genome and the 129-derived Fcγr2b flanking region cause loss of tolerance. The contribution to the development of autoimmune disease by the resulting autoreactive B cells is amplified by the absence of FcγRIIB, culminating in lethal lupus. In the presence of the Yaa lupus-susceptibility locus, FcγRIIBB6−/− mice do develop lethal lupus, confirming that FcγRIIB deficiency only amplifies spontaneous autoimmunity determined by other loci.