bluuebunny/crossref_metadata_2025_split huggingface dataset:
Using the TBX5 transcription factor to grow and sculpt the heart
Authors: Hatcher Cathy J. , McDermott Deborah A. 
Published: May 2006

AbstractTBX5 mutations cause the cardiac and limb defects of the autosomal dominant Holt–Oram syndrome (HOS). We have explored the role of the TBX5 transcription factor during cardiogenesis and have elucidated some of its functions in regulating myocardial cell proliferation and proepicardial cell migration. Our identification of TBX5 mutations has enabled us to offer genetic testing for diagnosis of HOS in patients and also to perform preimplantation genetic diagnosis on blastocysts for couples desiring to have a child unaffected by HOS. We hope that our genetic testing approach will serve as a paradigm for mutation screening in other inherited syndromes. © 2006 Wiley‐Liss, Inc.
Blepharophimosis‐mental retardation (BMR) syndromes: A proposed clinical classification of the so‐called Ohdo syndrome, and delineation of two new BMR syndromes, one X‐linked and one autosomal recessive
Authors: Verloes Alain , Bremond‐Gignac Dominique , Isidor Bertrand , David Albert , Baumann Clarisse , Leroy Marie‐Anne , Stevens René , Gillerot Yves , Héron Delphine , Héron Bénédicte , Benzacken Brigitte , Lacombe Didier , Brunner Han , Bitoun Pierre 
Published: May 2006

AbstractWe report on 11 patients from 8 families with a blepharophimosis and mental retardation syndrome (BMRS) phenotype. Using current nosology, five sporadic patients have Ohdo syndrome, associated with congenital hypothyroidism in two of them (thus also compatible with a diagnosis of Young‐Simpson syndrome). In two affected sibs with milder phenotype, compensated hypothyroidism was demonstrated. In another family, an affected boy was born to the unaffected sister of a previously reported patient. Finally, in the last sibship, two affected boys in addition had severe microcephaly and neurological anomalies. A definitive clinical and etiologic classification of BMRS is lacking, but closer phenotypic analysis should lead to a more useful appraisal of the BMRS phenotype. We suggest discontinuing the systematic use of the term “Ohdo syndrome” when referring to patients with BMRS. We propose a classification of BMRS into five groups: (1) del(3p) syndrome, (possibly overlooked in older reports); (2) BMRS, Ohdo type, limited to the original patients of Ohdo; (3) BMRS SBBYS (Say‐Barber/Biesecker/Young‐Simpson) type, with distinctive dysmorphic features and inconstant anomalies including heart defect, optic atrophy, deafness, hypoplastic teeth, cleft palate, joint limitations, and hypothyroidism. BMRS type SBBYS is probably an etiologically heterogeneous phenotype, as AD and apparently AR forms exist; (4) BMRS, MKB (Maat–Kievit–Brunner) type, with coarse, triangular face, which is probably sex‐linked; (5) BMRS V (Verloes) type, a probable new type with severe microcephaly, hypsarrhythmia, adducted thumbs, cleft palate, and abnormal genitalia, which is likely autosomal recessive. Types MKB and V are newly described here. © 2006 Wiley‐Liss, Inc.
Relationship between the absorption spectra and spatial hindrance in starch graft copolymers
Authors: Fu Zheng Sheng , Xue Hua Li , Wang Chang Qing , Liu Mao Dong , Yuan Li 
Published: May 2006

AbstractThe photochemical trans–cis isomerization of graft copolymers of azo dyes onto starch was studied in liquids spectrophotometrically. The relationship between the absorption spectra and spatial hindrance was investigated. The experimental results showed that the structure of the graft copolymers had a marked influence on photoisomerization. The smaller the spatial hindrance was, the easier the photoisomerization was. The copolymers showed better photoisomerization than their corresponding monomers, and this showed that the backbone of starch in the main chain influenced not the maximum‐wavelength position of the copolymers but the retroactive structure of the copolymers and favored their photoisomerization. In addition, the thermal cis–trans isomerization rate of the graft copolymers was studied in various polar solvents. The results suggested that the solvent polarity had a large effect on the thermal back‐isomerization. © 2006 Wiley Periodicals, Inc. J Appl Polym Sci 101: 2123–2126, 2006
eJournal interface can influence usage statistics: Implications for libraries, publishers, and Project COUNTER
Authors: Davis Philip M. , Price Jason S. 
Published: May 2006

AbstractThe design of a publisher's electronic interface can have a measurable effect on electronic journal usage statistics. A study of journal usage from six COUNTER‐compliant publishers at 32 research institutions in the United States, the United Kingdom, and Sweden indicates that the ratio of PDF to HTML views is not consistent across publisher interfaces, even after controlling for differences in publisher content. The number of full‐text downloads may be artificially inflated when publishers require users to view HTML versions before accessing PDF versions or when linking mechanisms, such as CrossRef, direct users to the full text rather than the abstract of each article. These results suggest that usage reports from COUNTER‐compliant publishers are not directly comparable in their current form. One solution may be to modify publisher numbers with “adjustment factors” deemed to be representative of the benefit or disadvantage due to its interface. Standardization of some interface and linking protocols may obviate these differences and allow for more accurate cross‐publisher comparisons.
Commonalities in compensation
Authors: Birchler James A. , Fernandez Harvey R. , Kavi Harsh H. 
Published: May 2006

AbstractThe sex chromosomes of many species differ in dosage but the total gene expression output is similar, a phenomenon referred to as dosage compensation. Previously, diverse mechanisms were postulated to account for compensation in distantly related taxa. However, two recent papers present evidence that dosage compensation in Drosophila, mammals and nematodes share the property that there is an approximately two‐fold upregulation of the single active X chromosome in each case.1,2 The results suggest that a common mechanism might operate in these different cases. BioEssays 28: 565–568, 2006. © 2006 Wiley Periodicals, Inc.
Comparing the Areas Under Two Correlated ROC Curves: Parametric and Non‐Parametric Approaches
Authors: Molodianovitch Katy , Faraggi David , Reiser Benjamin 
Published: May 2006

AbstractIn order to compare the discriminatory effectiveness of two diagnostic markers the equality of the areas under the respective Receiver Operating Characteristic Curves is commonly tested. A non‐parametric test based on the Mann‐Whitney statistic is generally used. Weiand et al. (1989) present a parametric test based on normal distributional assumptions. We extend this test using the Box‐Cox power family of transformations to non‐normal situations. These three test procedures are compared in terms of significance level and power by means of a large simulation study. Overall we find that transforming to normality is to be preferred. An example of two pancreatic cancer serum biomarkers is used to illustrate the methodology. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Recombinant interferon γ1b immune enhancement in 20 patients with hematologic malignancies and systemic opportunistic infections treated with donor granulocyte transfusions
Authors: Safdar Amar , Rodriguez Gilhen H. , Lichtiger Benjamin , Dickey Burton F. , Kontoyiannis Dimitrios P. , Freireich Emil J. , Shpall Elizabeth J. , Raad Issam I. , Kantarjian Hagop M. , Champlin Richard E. 
Published: May 2006

AbstractBACKGROUNDThe response to antifungal therapy alone often is suboptimal in patients with cancer who have therapy‐refractory neutropenia, and even donor‐derived granulocyte transfusions (GTX) are not always successful. The authors evaluated the safety and efficacy of immune enhancement using recombinant interferon γ1b (rIFN‐γ1b) in patients with cancer who received GTX for refractory, systemic, opportunistic infections.METHODSTwenty recipients of high‐dose donor GTX (≈5.5 × 1010 neutrophils per transfusion) who had received concurrent rIFN‐γ1b between October 2001 and December 2004 were evaluated retrospectively.RESULTSThe median age (± standard deviation [SD]) was 45 ± 17 years. Ten patients (50%) were men, 17 patients (85%) had leukemia, and 3 patients (15%) had myelodysplastic syndrome. The median ± SD Acute Physiology and Chronic Health Evaluation II score was 15 ± 4 (range, 7‐22). Most patients (n = 18 patients; 90%) had recurrent or refractory cancer. In 6 patients (30%) who received allogeneic hematopoietic stem cell transplantation, GTX plus rIFN‐γ1b was given a median ± SD of 26 ± 100 days (range, 12‐372 days) after transplantation. Seventeen patients (85%) had neutropenia during GTX therapy. Five patients (25%) had possible invasive fungal infection, 3 patients (15%) had probable invasive fungal infection, and 11 patients (55%) had proven invasive fungal infection. One patient (5%) had refractory Pseudomonas aeruginosa sepsis. Eight patients (40%) received corticosteroids during GTX plus rIFN‐γ1b therapy. Patients received a median ± SD of 8 ± 7 GTX doses (range, 4‐28 doses) and 9 ± 7 rIFN‐γ1b doses (range, 1‐28 doses), for a mean ± SD cumulative dose (CD) of 400 ± 2621 μg. Other concomitant cytokines were granulocyte‐colony stimulating factor (12 ± 3 doses; CD, 6720 ± 4721 μg) in 15 patients (75%) and granulocyte‐macrophage–colony stimulating factor (12 ± 9 doses; CD, 4750 ± 4410 μg) in 14 patients (70%). Four patients (20%) developed fever, and 2 patients (10%) developed skin rashes. Reversible liver dysfunction (n = 3 patients; 15%) and tachycardia (n = 1 patients; 5%) were considered rIFN‐γ1b‐associated adverse reactions; whereas, in 1 patient (5%), transient dyspnea was attributed to GTX. Four weeks after therapy started, 9 patients (45%) had complete or partial resolution of infection; and, in another 3 patients (15%), the invasive fungal infection had become stable.CONCLUSIONSThe current results indicated that no serious adverse events were associated with rIFN‐γ1b immune enhancement in patients with systemic opportunistic infections who received donor GTX therapy. Cancer 2006. © 2006 American Cancer Society.
Developing LHCb Grid software: experiences and advances
Authors: Stokes‐Rees I. , Tsaregorodtsev A. , Garonne V. , Graciani R. , Sanchez M. , Frank M. , Closier J. 
Published: May 2006

AbstractThe LHCb Grid software has been used for two Physics Data Challenges, with the latter producing over 98 TB of data and consuming over 650 processor‐years of computing power. This paper discusses the experience of developing a Grid infrastructure, interfacing to an existing Grid (LCG) and traditional computing centres simultaneously, running LHCb experiment software and jobs on the Grid, and the integration of a number of new technologies into the Grid infrastructure. Our experience and utilization of the following core technologies will be discussed: OGSI, XML‐RPC, Grid services, LCG middleware and instant messaging. Specific attention will be given to analysing the behaviour of over 100 000 jobs executed through the LCG Grid environment, providing insight into the performance, failure modes and scheduling efficiency over a period of several months for a large computational Grid incorporating over 40 sites and thousands of nodes. © Crown copyright 2006. Reproduced with the permission of Her Majesty's Stationery Office. Published by John Wiley & Sons, Ltd.
Performance and effectiveness trade‐off for checkpointing in fault‐tolerant distributed systems
Authors: Katsaros Panagiotis , Angelis Lefteris , Lazos Constantine 
Published: May 2006

AbstractCheckpointing has a crucial impact on systems' performance and fault‐tolerance effectiveness: excessive checkpointing results in performance degradation, while deficient checkpointing incurs expensive recovery. In distributed systems with independent checkpoint activities there is no easy way to determine checkpoint frequencies optimizing response‐time and fault‐tolerance costs at the same time. The purpose of this paper is to investigate the potentialities of a statistical decision‐making procedure. We adopt a simulation‐based approach for obtaining performance metrics that are afterwards used for determining a trade‐off between checkpoint interval reductions and efficiency in performance. Statistical methodology including experimental design, regression analysis and optimization provides us with the framework for comparing configurations, which use possibly different fault‐tolerance mechanisms (replication‐based or message‐logging‐based). Systematic research also allows us to take into account additional design factors, such as load balancing. The method is described in terms of a standardized object replication model (OMG FT‐CORBA), but it could also be applied in other (e.g. process‐based) computational models. Copyright © 2006 John Wiley & Sons, Ltd.
Nanobiotechnology: it's a small world after all
Authors: Moos Walter H. , Barry Stephen 
Published: May 2006

AbstractNanobiotechnology is the latest technology to provide disruptive—quantum leap—opportunities in drug development research. Examples of the utility of this approach are found increasingly in the scientific literature and the lay press. Whether for therapeutics, diagnostics, drug delivery, targeting, or some other product category, the potential for nanotechnology to revolutionize medicine is quite high. A number of organizations have chosen to focus their nanotechnology efforts on cancer. This is an exciting area that should benefit from an increasing number of collaborations of interdisciplinary experts to fabricate and characterize novel and enabling nanoparticulate technologies. Drug Dev. Res. 67:1–3, 2006.
Amphiphilic block copolymer micelles for nanoscale drug delivery
Authors: Kwon Glen S. , Forrest M. Laird 
Published: May 2006

AbstractAmphiphilic block copolymers can assemble into supramolecular core‐shell structures, termed ABC micelles, that have proven utility in drug delivery, particularly for drug solubilization. Several examples have entered clinical trials, attesting to the biocompatibility of ABCs and the potential advantages for drug delivery, e.g., low toxicity relative to Cremophor® EL, a surfactant commonly used for drug solubilization. Several examples of ABC micelles demonstrate potential for prolonged circulation in blood. Coupled with novel strategies toward controlled release of drug, nanoscale ABC micelles have tremendous potential for the targeting of antitumor agents, many of which are poorly water soluble and possess dose‐limiting toxicity. Drug Dev. Res. 67:15–22, 2006. © 2006 Wiley‐Liss, Inc.
Colloidal gold nanoparticles: a novel nanoparticle platform for developing multifunctional tumor‐targeted drug delivery vectors
Authors: Paciotti Giulio F. , Kingston David G.I. , Tamarkin Lawrence 
Published: May 2006

AbstractNanotechnology applied to biological problems represents an emerging field with the potential to offer extremely sensitive diagnostics and targeted cancer therapies. However, to achieve these goals, nanoparticle delivery systems must outwit the many barriers that are intrinsic to the body's defenses, as well as those that develop during the growth and progression of tumors. The science is advancing and, for example, true nanoscale tumor‐targeted drug delivery vectors are now able to reduce the likelihood of opsonization in the bloodstream and uptake by the reticuloendothelial system. Other advances hold promise for delivering multiple therapeutic agents to non‐homogeneous populations of cancer cells in solid tumors. We briefly summarize herein our attempts to build such multifunctional nanotherapeutics using colloidal gold nanoparticles. Specifically we discuss the development of colloidal gold‐based drugs that are designed to target the delivery of TNF and paclitaxel to solid tumors. Drug Dev. Res. 67:47–54, 2006. © 2006 Wiley‐Liss, Inc.
Discrete thermally responsive hydrogel‐coated gold nanoparticles for use as drug‐delivery vehicles
Authors: Kim Jun‐Hyun , Randall Lee T. 
Published: May 2006

AbstractThis report describes the development of optically responsive gold nanoparticles (∼60 nm in diameter) coated with a thermally responsive biocompatible hydrogel overlayer (20–90 nm thick). The hydrogel consists of a mixture of N‐isoproprylacrylamide and acrylic acid; this copolymer can be tailored to exhibit a lower critical solution temperature (LCST) slightly above physiological temperature. When the temperature is raised above the LCST, the hydrogel polymer shrinks dramatically; in contrast, when the temperature is lowered below the LCST, the hydrogel expands to a fully swollen structure. These hybrid nanoparticles were designed for the purpose of developing an optically modulated drug‐delivery system that responds to ambient changes in temperature. Specifically, drug‐impregnated hydrogel coatings can be photothermally activated by exposure to light that can be absorbed by the plasmon resonance of the gold nanoparticle cores. The studies described here demonstrate that these new hybrid core‐shell nanoparticles can be reproducibly prepared by surfactant‐free emulsion polymerization (SFEP) and that their structural responses to external stimuli are consistent with our objective of photothermal drug delivery. Drug Dev. Res. 67:61–69, 2006. © 2006 Wiley‐Liss, Inc.
Targeted molecular brachytherapy
Authors: Mayers George L. 
Published: May 2006

AbstractA new approach for the treatment of solid tumor cancers that utilizes aspects of bionanotechnology to deposit a short‐lived radioisotope throughout the tumor mass is described. Selectively Targeted Amplified Radiotherapy (STAR) is a multi‐step therapy in which an insoluble nano‐platform is first built inside neoplastic cells from a soluble precursor. The nano‐platform is then released into the extra‐cellular space throughout the tumor by disruption of some of the neoplastic cells. The nano‐platform is constructed so that it can selectively attach a reagent incorporating an exogenous enzyme, thereby transforming the insoluble nano‐platform into a nano‐factory. Finally, the enzymatic activity of the nano‐factory is used to convert a soluble radioactive substrate into a water‐insoluble nano‐precipitate throughout the tumor. The remaining soluble radiochemical is cleared from the body while the radioactive nano‐precipitate remains throughout the tumor, irradiating the surrounding tumor cells and providing what can be called molecular brachytherapy. This report discusses how the STAR technology overcomes tumor heterogeneity and metastases, the two major obstacles to successful treatment of solid tumor cancers. Drug Dev. Res. 67:94–106, 2006. © 2006 Wiley‐Liss, Inc.
Bmp2 and Gata4 function additively to rescue heart tube development in the absence of retinoids
Authors: Ghatpande Satish , Brand Thomas , Zile Maija , Evans Todd 
Published: May 2006

AbstractWe used the vitamin A–deficient (VAD) quail model to investigate the retinoid‐dependent mechanism that regulates heart tube development. We showed previously that decreased levels of Gata4 in cardiogenic mesoderm and endoderm correlate with the cardiomyopathy caused by VAD, but that this could be rescued by transplanting normal anterior endoderm. Bmp2 is a known cardiogenic factor that is expressed normally in lateral plate mesoderm and cardiac‐associated pharyngeal endoderm. Here we show that (like Gata4) transcripts encoding Bmp2 and BMP‐dependent signaling activity are decreased throughout the heart‐forming region of the VAD embryo. Addition of Bmp2 protein or forced expression of Gata4 in cultured VAD embryos leads to a partial rescue of the cardiomyopathy, and addition of both Bmp2 and Gata4 has an additive positive effect. Our data are consistent with a requirement for retinoid signaling to maintain expression of Bmp2, which regulates Gata4, and in addition acts with Gata4 to regulate genes important for normal morphogenesis of the primitive heart tube. Developmental Dynamics 235:2030–2039, 2006. © 2006 Wiley‐Liss, Inc.
Attenuation of retinal vascular development and neovascularization in transgenic mice over‐expressing thrombospondin‐1 in the lens
Authors: Wu Zhifeng , Wang Shoujian , Sorenson Christine M. , Sheibani Nader 
Published: May 2006

AbstractThrombospondin‐1 (TSP1) is an endogenous inhibitor of angiogenesis and induces endothelial cell (EC) apoptosis. To study the role TSP1 plays during vascular development and neovascularization, we assessed the effects of ectopic TSP1 expression in the lens on retinal vascularization in transgenic mice. The TSP1 over‐expressing mice showed abnormalities in the development of retinal vasculature. There was a dramatic decrease in the density of superficial and deep vascular plexuses of the retina in transgenic mice. The retinal vessels in TSP1 transgenic mice also appeared nonuniform and abnormal in maturation. We detected an increase in the number of EC undergoing apoptosis, which was compensated, in part, by an increase in cell proliferation in retinal vasculature of TSP1 transgenic mice. The TSP1 transgenic mice also exhibited increased levels of vessel obliteration and a limited preretinal neovascularization during oxygen‐induced ischemic retinopathy (OIR). Our results indicate increased expression of TSP1 attenuates normal retinal vascularization and preretinal neovascularization during OIR. Therefore, modulation of TSP1 expression may provide an effective mechanism for regulation of ocular angiogenesis. Developmental Dynamics 235:1908–1920, 2006. © 2006 Wiley‐Liss, Inc.
Dicopper(II) Complexes with the Enantiomers of a Bidentate Chiral Reduced Schiff Base: Inclusion of Chlorinated Solvents and Chiral Recognition of1,2‐Dichloroethane Rotamers in the Crystal Lattice
Authors: Muppidi Vamsee Krishna , Pal Samudranil 
Published: May 2006

AbstractBisphenoxo‐bridged dicopper(II) complexes [Cu2Ln2Cl2] {1 (n = 1) and 2 (n = 2)} with the N,O‐donor reduced Schiff bases N‐(2‐hydroxybenzyl)‐(R)‐α‐methylbenzylamine (HL1) and N‐(2‐hydroxybenzyl)‐(S)‐α‐methylbenzylamine (HL2) have been synthesised and characterised. In both 1 and 2, the bidentate chiral ligands coordinate the metal centres through the secondary amine N atom and the bridging phenolate O atom. The chloride ion occupies the fourth coordination site and completes a slightly distorted square‐planar NO2Cl environment around each copper(II) centre. Magnetic susceptibility measurements in the solid state suggest a strong antiferromagnetic interaction between the metal centres in both complexes. Both 1 and 2 readily form 1:1 host‐guest compounds with chlorinated solvents such as CH2Cl2, CHCl3 and Cl(CH2)2Cl. All the host‐guest compounds crystallise in noncentrosymmetric space groups. 1·CH2Cl2 and 2·CH2Cl2 crystallise in the P21 space group while 1·CHCl3, 2·CHCl3, 1·Cl(CH2)2Cl and 2·Cl(CH2)2Cl crystallise in the P212121 space group. In these inclusion crystals, the C–H···Cl interactions between the guest and the host molecules are primarily responsible for enclatheration of the chloroalkane molecules. In the case of CH2Cl2, one of its Cl atoms acts as the acceptor. On the other hand, for CHCl3 and Cl(CH2)2Cl, the metal coordinated Cl atom of the host complex acts as the acceptor. The structures of 1·(P)‐Cl(CH2)2Cl and 2·(M)‐Cl(CH2)2Cl provide rare examples for chiral recognition of the right handed (P) and the left handed (M) gauche forms of Cl(CH2)2Cl in molecular assemblies. (© Wiley‐VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2006)