bluuebunny/crossref_metadata_2025_split huggingface dataset:
The role of junctional adhesion molecule C (JAM‐C) in acute pancreatitis
Authors: Vonlaufen A , Aurrand‐Lions M , Pastor CM , Lamagna C , Hadengue A , Imhof BA , Frossard J‐L 
Published: June 2006

AbstractThe recruitment of inflammatory cells contributes significantly to tissue injury in acute pancreatitis. This process implies several molecular interactions between circulating and endothelial cells. The adhesion molecule junctional adhesion molecule C (JAM‐C) is involved in leukocyte transendothelial migration and it can form homophilic (JAM‐C/JAM‐C) and heterophilic interactions with the leukocyte integrin αMβ2. In this study, the effect of early administration of monoclonal antibodies directed against JAM‐C in cerulein‐induced acute pancreatitis was assessed. This reagent significantly blocked influx of leukocytes, release of serum amylase, secretion of inflammatory cytokines, and acinar cell necrosis. These effects were rapid and protected against tissue injury throughout the duration of the model. Conversely, cerulein‐induced acute pancreatitis was more severe in transgenic mice overexpressing JAM‐C on endothelial cells under the control of the Tie2 promoter. It is proposed that JAM‐C expressed by endothelial cells contributes to the pathophysiology of acute pancreatitis and could be considered a target for clinical applications. Copyright © 2006 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Protection against chemotherapy induced mucositis by TGF‐β2 in childhood cancer patients: Results from a randomized cross‐over study
Authors: de Koning Barbara A.E. , Philipsen‐Geerling Bertine , Hoijer Maarten , Hählen Karel , Büller Hans A. , Pieters Rob 
Published: June 2006

AbstractBackgroundMucositis is one of the most frequent and severe side‐effect of chemotherapy in childhood‐cancer patients for which there is no prophylaxis available. The efficacy and feasibility of a TGF‐β2‐enriched feeding for preventing oral and gastro‐intestinal‐mucositis in childhood‐cancer patients were studied.ProcedureThe study was designed as a two‐period cross‐over, randomized, double‐blinded, placebo, controlled trial. Patients who had a high risk for developing mucositis and who would receive two comparable cycles of chemotherapy were eligible for the study. During one cycle of chemotherapy, TGF‐β2‐enriched feeding was administered; during the other, a “placebo” (not enriched) feeding was used. WHO toxicity scales of diarrhea, oral mucositis, fever, anal lesions and nausea/vomiting were scored daily. In addition, the incidence of occurrence of blood cultures, antibiotic therapy, and interventions or diagnostics related to mucositis were measured.ResultsThe feasibility of the study was good: 83% of the patients completed two cycles and 86% of the study‐feeding was effectively consumed. Administration of TGF‐β2 was safe as serum TGF‐β2 did not increase, and renal and liver function were not affected during TGF‐β2 consumption compared to normal feeding. Differences in toxicity, scored during the whole observation period and the number of days with WHO 3/4 toxicity, were not significantly different between cycles with TGF‐β2 enriched and normal feeding.ConclusionsTGF‐β2 administration via feeding is well tolerated and safe. Although this study might have had limitations to show potential benefit of TGF‐β2, it does not provide evidence that TGF‐β2 decreases the incidence or degree of mucositis induced by combination chemotherapy in childhood‐cancer patients. Pediatr Blood Cancer 2007;48:532–539. © 2006 Wiley‐Liss, Inc.
First‐ and second‐trimester Down syndrome screening markers in pregnancies achieved through assisted reproductive technologies (ART): a FASTER trial study
Authors: Lambert‐Messerlian G. , Dugoff L. , Vidaver J. , Canick J. A. , Malone F. D. , Ball R. H. , Comstock C. H. , Nyberg D. A. , Saade G. , Eddleman K. , Klugman S. , Craigo S. D. , Timor‐Tritsch I. E. , Carr S. R. , Wolfe H. M. , D'Alton M. E. 
Published: June 2006

AbstractObjectiveTo determine whether first‐ and second‐trimester Down syndrome screening markers and screen‐positive rates are altered in pregnancies conceived using assisted reproductive technologies (ARTs).MethodsART pregnancies in the multicenter FASTER trial were identified. Marker levels were evaluated for five types of ART: in vitro fertilization with ovulation induction (IVF‐OI), IVF with OI and egg donation (IVF‐OI‐ED), IVF with ED (IVF‐ED), and intrauterine insemination with OI (IUI‐OI) or without OI (IUI). Each group was compared to non‐ART controls using Mann–Whitney U analysis.ResultsFirst‐trimester marker levels were not significantly different between ART and control pregnancies, with the exception of reduced PAPP‐A levels in the IUI‐OI group. In contrast, second‐trimester inhibin A levels were increased in all ART pregnancies, estriol was reduced and human chorionic gonadotropin (hCG) was increased in IVF and IUI pregnancies without ED, and alpha‐fetoprotein (AFP) was increased in ED pregnancies. Second‐trimester screen‐positive rates were significantly higher than expected for ART pregnancies, except when ED was used.ConclusionsThese data show that ART significantly impacts second‐, but not first‐, trimester markers and screen‐positive rates. The type of adjustment needed in second‐trimester screening depends on the particular type of ART used. Copyright © 2006 John Wiley & Sons, Ltd.
Matrix‐assisted laser desorption/ionization time‐of‐flight mass spectroscopic analysis of telechelic polythiourethanes obtained by the cationic ring‐opening polymerization of six‐membered cyclic thiourethane
Authors: Nagai Daisuke , Sato Masato , Ochiai Bungo , Endo Takeshi 
Published: June 2006

AbstractA matrix‐assisted laser desorption/ionization time‐of‐flight (MALDI‐TOF) mass spectroscopy analysis of polythiourethanes obtained by the cationic ring‐opening polymerization of a six‐membered cyclic thiourethane [3‐benzyltetrahydro‐1,3‐oxazine‐2‐thione (BTOT)] is described. A MALDI‐TOF mass spectrum of a polymer obtained by the polymerization of BTOT with boron trifluoride etherate (BF3OEt2) as the initiator in nitrobenzene at 50 °C for 24 h followed by an end‐capping reaction with diethyldithiocarbamic acid diethylammonium salt showed a series of well‐resolved signals that were assignable to polythiourethanes possessing an amino group at the initiating end and a diethyldithiocarbamate group at the terminating end. In comparison with the MALDI‐TOF mass spectra of polymers obtained by polymerization with trifluoromethanesulfonic acid or methyl trifluoromethanesulfonate, the plausible initiating species in the polymerization with BF3OEt2 was estimated to be a proton, which successively eliminated carbonyl sulfide to produce a secondary amine group at the initiating end. The secondary amine group in the obtained telechelic polymer was converted to a tertiary amine group by a reaction with benzyl bromide in the presence of triethylamine, and this was confirmed by MALDI‐TOF mass spectroscopy. Furthermore, a telechelic polymer with a pyrrole end group was successfully synthesized by the end‐capping reaction of the growing species in the polymerization of BTOT with sodium 1‐pyrrolecarbodithioate. © 2006 Wiley Periodicals, Inc. J Polym Sci Part A: Polym Chem 44: 4281–4289, 2006
Charge density effects in salt‐free polyelectrolyte solution rheology
Authors: Dou Shichen , Colby Ralph H. 
Published: June 2006

AbstractSolution rheology of 2‐vinyl pyridine andN‐methyl‐2‐vinyl pyridinium chloride random copolymers in ethylene glycol was studied over wide ranges of concentration and effective charge. The fraction of quaternized monomers α and the fraction of monomers bearing an effective chargefof these copolymers were measured using counterion titration and dielectric spectroscopy, respectively. Ethylene glycol is a good solvent for neutral poly(2‐vinyl pyridine), with very few ionic impurities. The viscosity η and relaxation time τ of dilute and semidilute unentangled solutions exhibit the scaling with concentration and effective charge expected by the Dobrynin model. Reduced viscosity data are independent of concentration in dilute solution, giving an intrinsic viscosity that depends on effective charge, and the experimental data obey the Fuoss law in the semidilute unentangled regime. Scaling concentration with the overlap concentration (c/c*) reduces these data to common curves, andc*∼f−12/7as predicted by the Dobrynin model, wherefis the fraction of monomers bearing an effective charge. While the overlap concentration depends strongly on effective charge until counterion condensation occurs, the entanglement concentrationceis surprisingly insensitive to effective charge, indicating that entanglement effects are not understood using the Dobrynin model. The terminal modulusG= η/τ depends only on the number density of chainsG=ckT/Nforc* <c<ce, andG∼c3/2forc>ceindependent of the effective charge. © 2006 Wiley Periodicals, Inc. J Polym Sci Part B: Polym Phys 44: 2001–2013, 2006
Atomic structure and relative stability of gallium and nitrogen interstitials in GaN [0001] grain boundaries
Authors: Béré A. , Belabbas I. , Petit S. , Nouet G. , Ruterana P. , Chen J. , Koulidiati J. 
Published: June 2006

AbstractThe atomic and electronic structures of gallium and nitrogen interstitial created at the 8‐interface of the (2$\bar 5$30) Σ = 19 (θ = 13.2°) tilt grain boundary in GaN were investigated by density‐functional based tight‐binding calculations. We found that the structure of lowest energy of the Ga‐ or N‐interstitial boundary is formed by a mixture of 6‐ and 5/7‐atom rings (Gai‐ or Ni‐interface) which is comparable to that of the 5/7‐interface. The calculations show that the non stoichiometric Gai‐interface is energetically more stable than the stoichiometric 5/7‐interface, suggesting that both configurations may exist. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Influence of enhanced temperature and pressure on structural transformations in pre‐annealed Cz‐Si
Authors: Ciosek Jerzy , Misiuk Andrzej , Surma Barbara , Shchennikov Vladimir V. 
Published: June 2006

AbstractOxygen‐related defects in oxygen‐containing Czochralski silicon (Cz‐Si) subjected to one or 4‐steps pre‐annealing at 720–1000 K under 105 Pa and next treated at 1170–1400 K (HT) under high hydrostatic pressure (HP, up to 1.2 GPa) were investigated by photoluminescence (PL) and microhardness measurements as well as by spectroscopic reflectrometry. Microstructure of HT‐HP treated Cz‐Si is dependent on nucleation centres for oxygen precipitation created by pre‐annealing and on applied pressure. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Behaviour of the AlN cap during GaN implantation of rare earths and annealing
Authors: Gloux Florence , Wójtowicz Tomasz , Ruterana Pierre , Lorenz K. , Alves E. 
Published: June 2006

AbstractThe structural evolution of 10 nm AlN caps grown on GaN layers has been investigated by transmission electron microscopy (TEM) and scanning electron microscopy (SEM) after growth, and implantation by rare earths ions and annealing at high temperature. The AlN cap relaxation is shown to take place by pinholes formation. During annealing at 1300 °C, pinhole‐free areas of the AlN cap protect the GaN layer and allow efficient optical activation of the rare earths dopants. Through pinholes, an explosive dissociation of the GaN layer occurs. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Feasibility of different mass spectrometric techniques and programs for automated metabolite profiling of tramadol in human urine
Authors: Hakala Kati S. , Kostiainen Risto , Ketola Raimo A. 
Published: June 2006

AbstractThe purpose of the study was to determine the advantages of different mass spectrometric instruments and commercially available metabolite identification programs for metabolite profiling. Metabolism of tramadol hydrochloride and the excretion of it and its metabolites into human urine were used as a test case because the metabolism of tramadol is extensive and well known. Accurate mass measurements were carried out with a quadrupole time‐of‐flight mass spectrometer (Q‐TOF) equipped with a LockSpray dual‐electrospray ionization source. A triple quadrupole mass spectrometer (QqQ) was applied for full scan, product ion scan, precursor ion scan and neutral loss scan measurements and an ion trap instrument for full scan and product ion measurements. The performance of two metabolite identification programs was tested. The results showed that metabolite programs are time‐saving tools but not yet capable of fully automated metabolite profiling. Detection of non‐expected metabolites, especially at low concentrations in a complex matrix, is still almost impossible. With low‐resolution instruments urine samples proved to be challenging even in a search for expected metabolites. Many false‐positive hits were obtained with the automated searching and manual evaluation of the resulting data was required. False positives were avoided by using the higher mass accuracy Q‐TOF. Automated programs were useful for constructing product ion methods, but the time‐consuming interpretation of mass spectra was done manually. High‐quality MS/MS spectra acquired on the QqQ instrument were used for confirmation of the tramadol metabolites. Although the ion trap instrument is of undisputable benefit in MSn, the low mass cutoff of the ion trap made the identification of tramadol metabolites difficult. Some previously unreported metabolites of tramadol were found in the tramadol urine sample, and their identification was based solely on LC/MS and LC/MS/MS measurements. Copyright © 2006 John Wiley & Sons, Ltd.
Therapy through chaperones: Sense or antisense? Cystic fibrosis as a model disease
Authors: Amaral Margarida D. 
Published: June 2006

SummaryMassive production and accumulation of a single abnormal protein may constitute a major toxic burden for the cell and even compromise the organism's long‐term viability. Consequently, adaptation and survival have forced evolution to create ‘quality control’ mechanisms that detect, monitor, and often degrade such abnormally folded gene products, in which molecular chaperones are key players. Notwithstanding this, there are numerous examples of misfolded proteins which, in spite of being recognized as aberrant and efficiently discarded by cellular quality control, still retain some of the functional properties of their wild‐type counterparts, so that their maintenance in the cell would be beneficial for the organism. Herein are described the cellular roles of molecular chaperones and some new insights on the mechanisms by which they influence the development of human diseases caused by mutations that lead to protein misfolding. A special emphasis is given to cystic fibrosis, a classical genetic disorder resulting from the retention and degradation of a mutant, albeit functional, protein by the endoplasmic reticulum quality control. This particular system has been a good example to describe the mechanisms that are likely to be shared by a number of protein substrates, to define the common characteristics of the mutants, as well as to identify the mechanistic intervenients in their retention and degradation. Finally, new approaches aimed at correcting protein folding defects are discussed, including the potential of molecular chaperones (e.g., through RNA interference) as novel therapeutic targets, and the usage of chemical or pharmacological chaperones as new therapeutic agents.
Hepatocyte transplantation: Studies in preclinical models
Authors: Weber Anne , Mahieu‐Caputo Dominique , Hadchouel Michelle , Franco Dominique 
Published: June 2006

SummaryTransplantation of allogeneic or genetically modified autologous hepatocytes may be an alternative to whole‐liver transplantation for the treatment of hereditary metabolic liver diseases. Human hepatocytes have already been transplanted in patients, demonstrating the safety and feasibility of both approaches. Although a few cases of allogeneic transplantation have resulted in long‐term engraftment and function, only a partial and transient correction of the disease was achieved. This may partly result from a lack of proliferation of transplanted cells. In rodents, transplanted hepatocytes do not proliferate in adult quiescent livers and repopulate recipient livers only when they display a proliferative advantage over resident hepatocytes. Most of these models are not transposable to humans, however. Our aim is to develop preclinical approaches to hepatocyte transplantation in nonhuman primates. We have defined a strategy that increases the engraftment efficiency of transplanted hepatocytes by inducing their proliferation together with that of resident hepatocytes. We have also immortalized simian fetal hepatic progenitor cells and shown that these cells do not proliferate in situ after transplantation into the livers of immunodeficient mice. By contrast early human hepatoblasts repopulate mouse livers more efficiently. However, if we consider the number of cells to be transplanted (one to several billion), the means of expanding and differentiating stem or progenitor cells other than hepatocytes will have to be determined prior to envisaging treating patients.
Substrate reduction therapy of glycosphingolipid storage disorders
Authors: Aerts Johannes M. F. G. , Hollak Carla E. M. , Boot Rolf G. , Groener Johanna E. M. , Maas Mario 
Published: June 2006

SummaryIn the last 15 years enormous progress has been made regarding therapy of type I Gaucher disease, a severely disabling disorder characterized by intralysosomal storage of glucosylceramide in tissue macrophages. Effective enzyme replacement therapy of type I Gaucher disease, based on chronic intravenous administration of mannose‐terminated recombinant human glucocerebrosidase, has been available since 1990 and has been applied in several thousand patients without serious adverse effects. An alternative therapeutic approach, so‐called substrate reduction therapy, is based on partial reduction of the synthesis of glucosylceramide and hence of subsequent metabolites. Oral administration of an inhibitor of glucosylceramide synthesis (N‐butyldeoxynojirimycin, registered in Europe since 2002 as miglustat (Zavesca)), is effective in reversing clinical symptoms in type I Gaucher patients with mild to moderate disease manifestations. The growing long‐term experience with substrate reduction therapy indicates that this treatment is also without major adverse effects. Substrate reduction therapy, in conjunction with enzyme replacement therapy, may play an important role in the future clinical management of patients suffering from type I Gaucher disease. Clinical trials are under way that should reveal the value of substrate reduction for maintenance therapy of type I Gaucher disease and for treatment of neuronopathic variants of Gaucher disease, Niemann–Pick disease type C, late‐onset Tay–Sachs disease and Sandhoff disease.
β‐Galactosidase deficiency: An approach to chaperone therapy
Authors: Suzuki Yoshiyuki 
Published: June 2006

SummaryWe propose a new molecular therapeutic approach to lysosomal diseases with severe neurological manifestations. Some low‐molecular‐weight compounds, acting as competitive inhibitors of a lysosomal enzyme in vitro, were found to stabilize and restore catalytic activities of the enzyme molecule as a molecular chaperone. We started this trial first in Fabry disease (generalized vasculopathy) using galactose and 1‐deoxygalactonojirimycin, and then in β‐galactosidase deficiency disorders (β‐galactosidosis) with generalized neurosomatic and/or systemic skeletal manifestations (GM1‐gangliosidosis and Morquio B disease), using a newly developed chemical compound N‐octyl‐4‐epi‐β‐valienamine (NOEV). Administration of this chaperone compound resulted in elevation of intracellular enzyme activity in cultured fibroblasts from patients and genetically engineered model mice. In addition, substrate storage was improved after NOEV had been transported into the brain tissue via the blood–brain barrier. We hope this new approach (chemical chaperone therapy) will be useful for certain patients with β‐galactosidosis and potentially other lysosomal storage diseases with central nervous system involvement.
Anaplerotic diet therapy in inherited metabolic disease: Therapeutic potential
Authors: Roe Charles R. , Mochel Fanny 
Published: June 2006

SummaryBeginning with phenylketonuria, dietary therapy for inborn errors has focused primarily on the restriction of the precursor to an affected catabolic pathway in an attempt to limit the production of potential toxins. Anaplerotic therapy is based on the concept that there may exist an energy deficit in these diseases that might be improved by providing alternative substrate for both the citric acid cycle (CAC) and the electron transport chain for enhanced ATP production. This article focuses on this basic problem, as it may relate to most catabolic disorders, and provides our current experience involving inherited diseases of mitochondrial fat oxidation, glycogen storage, and pyruvate metabolism using the anaplerotic compound triheptanoin. The observations have led to a realization that ‘inter‐organ’ signalling and ‘nutrient sensors’ such as adenylate monophosphate mediated‐protein kinase (AMPK) and mTOR (mammalian target of rapamycin) appear to play a significant role in the intermediary metabolism of these diseases. Activated AMPK turns on catabolic pathways to augment ATP production while turning off synthetic pathways that consume ATP. Information is provided regarding the inter‐organ requirements for more normal metabolic function during crisis and how anaplerotic therapy using triheptanoin, as a direct source of substrate to the CAC for energy production, appears to be a more successful approach to an improved quality of life for these patients.
Newborn screening for medium‐chain acyl‐CoA dehydrogenase deficiency: A global perspective
Authors: Rhead William J. 
Published: June 2006

SummaryAs judged by tandem mass spectrometry blood spot screening, the incidence of medium‐chain acyl‐CoA dehydrogenase (MCAD) deficiency is 1:14 600 (CI 95%: 1:13 500–1:15 900) in almost 8.2 million newborns worldwide and is 2‐ to‐3 fold higher than that identified in the same populations after clinical presentation. In mass‐screened newborn populations, the 985A>G (K329E) mutation accounts for 54–90% of disease alleles, with homozygotes representing about 47–80% of MCAD deficiency cases. Worldwide, octanoylcarnitine levels are an effective primary screen for MCAD deficiency in newborns. Newborns homozygous for the 985A < G mutation have higher octanoylcarnitine levels than do those compound heterozygous for 985A < G and those with other genotypes. Time of sampling after birth also significantly affects octanoylcarnitine levels in MCAD‐deficient newborns. Tandem mass spectrometry newborn blood spot screening for MCAD deficiency is accurate and effective, reduces morbidity and mortality, and merits expansion to other populations worldwide.