bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cervico‐medullary desmoplastic infantile ganglioglioma: An unusual case with diffuse leptomeningeal dissemination at diagnosis
Authors: Milanaccio Claudia , Nozza Paolo , Ravegnani Marcello , Rossi Andrea , Raso Alessandro , Gambini Claudio , Luisa Garrè Maria , Pietsch Torsten 
Published: February 2005

AbstractDesmoplastic infantile ganglioglioma (DIG) is a rare intracranial tumor affecting newborns and infants, and generally arising in the supratentorial region. We report a case of an unusual DIG, arising at the cervico‐medullary junction, with diffuse leptomeningeal seeding at diagnosis that was treated with chemotherapy (CT) and delayed partial surgery. The case we describe demonstrates that DIG can occur in the subtentorial region and can show leptomeningeal dissemination like other low‐grade gliomas. © 2005 Wiley‐Liss, Inc.
Oxidase activity in cord blood neutrophils: A balance between increased membrane associated cytochrome b558 and deficient cytosolic components
Authors: Chudgar Urmish H. , Thurman Gail W. , Ambruso Daniel R. 
Published: February 2005

AbstractIntroductionNewborn infants are prone to develop life‐threatening pyogenic infections. Alterations in the function of neonatal phagocytes, including the activity of the neutrophil NADPH oxidase, have been suggested as one cause of increased susceptibility to such infections.MethodsIn the present study, comprehensive analysis of NADPH oxidase enzyme system was performed in cord blood neutrophils from vaginally and cesarean section (CS) delivered, healthy, full‐term infants.ResultsSuperoxide anion (O) production by intact neutrophils from cord blood in response to soluble stimuli was equal to or increased compared to levels generated by cells from adult controls. In the sodium dodecyl sulfate (SDS) cell‐free system, cytosol and plasma membrane from cord blood neutrophils generated O at comparable rates to subcellular fractions from healthy adults. However, mixing experiments demonstrated higher O generation with combination of cytosol from adult controls and membrane from cord blood neutrophils and lower O production with combination of cytosol from cord blood neutrophils and membrane from adult controls. Kinetic parameters for cord blood specimens were no different from those obtained for fractions from adult controls. Quantitative analysis of cytosolic components showed moderately reduced amount of p40‐phox, p47‐phox, and p67‐phox in neutrophils from cord blood. In contrast, cytochrome b558 content of plasma membrane of cord blood neutrophils was ∼2‐fold higher compared to adult controls.ConclusionThe normal to increased respiratory burst of intact cord blood neutrophils is the result of alterations to oxidase components: increased content of cytochrome b558 in the plasma membrane and decreased levels of cytosolic components p47‐phox, p67‐phox, and p40‐phox. © 2005 Wiley‐Liss, Inc.
New light‐colored poly(ether imide)s based on 2,5‐di‐tert‐butylhydroquinone bis(ether anhydride) and aromatic diamines
Authors: Yang Chin‐Ping , Hsu Mei‐Yuan 
Published: February 2005

AbstractA series of poly(ether imide)s (PEIs), IIIa–k, with light color and good physical properties were prepared from 1,4‐bis(3,4‐dicarboxypheoxy)‐2,5‐di‐tert‐butylbenzene dianhydride (I) with various aromatic diamines (IIa–k) via a conventional two‐stage procedure that included a ring‐opening polyaddition to yield poly(amic acid)s (PAA), followed by thermal imidization to the PEI. The intermediate PAA had inherent viscosities in the range of 1.00–1.53 dL g−1. Most of the PEIs showed excellent solubility in chlorinated solvents such as dichloromethane, chloroform, and m‐cresol, but did not easily dissolve in dimethyl sulfoxide and amide‐type polar solvents. The III series had tensile strengths of 96–116 MPa, an elongation at break of 7–8%, and initial moduli of 2.0–2.5 GPa. The glass‐transition temperatures (Tg) and softening temperatures (Ts's) of the III series were recorded between 232 and 285 °C and 216–279 °C, respectively. The decomposition temperatures for 10% weight loss all occurred above 511 °C in nitrogen and 487 °C in air. The III series showed low dielectric constants (2.71–3.54 at 1 MHz), low moisture absorption (0.18–0.66 wt %), and was light‐colored with a cutoff wavelength below 380 nm and a low yellow index (b*) values of 7.3–14.8. © 2005 Wiley Periodicals, Inc. J Polym Sci Part A: Polym Chem 43: 1270–1284, 2005
Effects of distortion–rotation of potential energy surfaces on absorption and resonance raman cross sections of trans‐stilbene molecule
Authors: Dehestani M. , Islampour R. 
Published: February 2005

AbstractThe time–correlation function formalism has been used to calculate resonance Raman cross sections and electronic absorption spectra of the trans‐stilbene molecule. The multidimensional time domain integrals that arise in these calculations have been evaluated for the cases in which electronic transitions take place between displaced, displaced–distorted, and displaced–distorted–rotated harmonic potential energy surfaces. The effects of distortions and rotations of potential energy surfaces on absorption, and resonance Raman cross sections are investigated quantitatively. The calculated absorption spectra and resonance Raman cross sections have been compared with the experimental results. Finally, resonance Raman excitation profiles have been simulated for five vibrational modes of trans‐stilbene, assuming the displaced–distorted–rotated harmonic oscillator model. © 2005 Wiley Periodicals, Inc. Int J Quantum Chem, 2005
Linear phosphorus–boron chains: model system with huge electronic first hyperpolarizability
Authors: Jacquemin Denis , Medved′ Miroslav , Perpète Eric A. 
Published: February 2005

AbstractThe longitudinal electronic static first hyperpolarizability of model linear phosphorus–boron chains is investigated using ab initio tools, including dynamic electron correlation corrections. The polymer presents an extremely large first hyperpolarizability (1700 × 10−30 esu per unit cell) that can be rationalized in terms of delocalization and asymmetry (unit cell and chain‐ends contributions). © 2005 Wiley Periodicals, Inc. Int J Quantum Chem, 2005
A shotgun tandem mass spectrometric analysis of phospholipids with normal‐phase and/or reverse‐phase liquid chromatography/electrospray ionization mass spectrometry
Authors: Houjou Toshiaki , Yamatani Kotoko , Imagawa Masayoshi , Shimizu Takao , Taguchi Ryo 
Published: February 2005

AbstractElectrospray ionization mass spectrometry is used in lipidomics studies. The present research established a top‐down liquid chromatography/electrospray ionization tandem mass spectrometry (LC/ESI‐MS/MS) shotgun analysis method for phospholipids (PLs) using a normal‐phase column or a C30 reverse‐phase column with the data‐dependent MS/MS scanning mode. A normal‐phase column can separate most of the major different classes of PLs. By using LC/ESI‐MS/MS with a normal‐phase column, approximately 50 molecular species were identified in a PL mixture from rat liver. When the reverse‐phase column was used, the PLs could be separated depending on their hydrophobicity, essentially the length of their fatty acyl chains and the number of unsaturated bonds in them. The LC/ESI‐MS/MS method using a C30 reverse‐phase column was applied to phosphatidylcholine (PC) and phosphatidylethanolamine (PE) mixtures as test samples. Molecular species with the same molecular mass but with different pairs of fatty acyl chains were separately identified. As a result, about 60 PC and 50 PE species were identified. PLs from rat liver were subjected to LC/ESI‐MS/MS using the C30 reverse‐phase column and about 110 molecular species were identified. Off‐line two‐dimensional LC/ESI‐MS/MS with the normal‐phase and C30 reverse‐phase columns allowed more accurate identification of molecular species by using one‐dimensional C30 reverse‐phase LC/ESI‐MS/MS analysis of the collected fractions. Copyright © 2005 John Wiley & Sons, Ltd.
Acetonitrile/water gas‐phase reaction products observed by use of atmospheric pressure chemical ionization: adducts formed with sulfonamides
Authors: Ross Charles W. , Coddington Arthur B. , Murphy Joan S. , Wisnoski David D. , Zartman C. Blair , Ramjit Harri G. 
Published: February 2005

AbstractElectrospray ionization (ESI) and atmospheric pressure chemical ionization (APCI) are the two most common mass spectrometric ionization methods used in the pharmaceutical industry. However, APCI analysis can sometimes lead to ambiguity in compound characterization and quantitation due to gas‐phase reactions occurring between acetonitrile and water in the plasma, and between these plasma‐generated compounds and the analyte. During the analysis of various sultams and sulfonamides we observed signals corresponding to m/z [M+44]+ and [M+60]+. Various solvent conditions and collisionally activated dissociation MSn experiments revealed that under the high‐energy plasma conditions of APCI, the acetonitrile/water solvent mixture reacts undergoing acid‐catalyzed hydrolysis producing acetamide, 59 Da. Further, the highly reactive 43 Da species ethanimine is also produced. These two compounds, normally not observed in APCI analysis, are stabilized by the sulfonamide and appear as adduct species in the mass spectra. The sulfone oxygens and the lone pair of electrons on the amide nitrogen play a role in stabilizing this adduct. Copyright © 2005 John Wiley & Sons, Ltd.
Selective detection of thiosulfate‐containing peptides using tandem mass spectrometry
Authors: Raftery Mark J. 
Published: February 2005

AbstractIncubation of proteins or peptides containing disulfide bonds (SS) with sodium sulfite (Na2SO3) cleaves SS bonds producing approximately equimolar amounts of free thiols (SH) and thiosulfates (SSO3H), a process known as sulfitolysis. Proteins and peptides containing thiosulfates were separated by reverse‐phase high‐performance liquid chromatography (RP‐HPLC) and characterized by mass spectrometry (MS) and peptide mapping. The mass of the thiosulfate‐containing peptide formed from oxidized insulin B chain was 3478.02 Da, 80 Da greater than the reduced peptide and corresponding precisely to addition of sulfur trioxide (SO3). Disulfide bond cleavage was also observed using RP‐HPLC and MS after incubation of the intramolecular homodimer of mouse S100A8 (mass 20614 Da). The mass of HPLC‐separated A8‐SH was 10308 Da, and 10388 Da for A8‐SSO3H. Loss of SO3 from multiply charged precursor ions was generally observed at elevated declustering potentials in the source region or within q2 at relatively low collision energies (∼20 V). The characteristic loss of SO3 at low collision energies preceded peptide backbone fragmentations at higher collision energies. Accurate mass measurement and charge‐state discrimination, using a hybrid quadrupole time‐of‐flight mass spectrometer, allowed specific detection of thiosulfate‐containing peptides. An information‐dependent acquisition method, where the switch criterion was loss of m/z 79.9568, specifically identified 11 thiosulfate‐containing peptides using nano‐LC/MS from a tryptic digest of bovine serum albumin (BSA). Copyright © 2005 John Wiley & Sons, Ltd.
Global and site‐specific detection of human integrin α5β1 glycosylation using tandem mass spectrometry and the StrOligo algorithm
Authors: Ethier Martin , Krokhin Oleg , Ens Werner , Standing Kenneth G. , Wilkins John A. , Perreault Hélène 
Published: February 2005

AbstractGlycans are oligosaccharides associated with proteins, and are known to confer specific functions and conformations on glycoproteins. As protein tridimensional structures are related to function, the study of glycans and their impact on protein folding can provide important information to the field of proteomics. The subdiscipline of glycomics (or glycoproteomics) is rapidly growing in importance as glycans in proteins have shown to be involved in protein–protein or protein–(drug, virus, antibody) interactions. Glycomics studies most often aim at identifying glycosylation sites, and thus are performed on deglycosylated proteins resulting in loss of site‐specific details concerning the glycosylation. In order to obtain such details by mass spectrometry (MS), either whole glycoproteins must be digested and analyzed as mixtures of peptides and glycopeptides, or glycans must be isolated from glycopeptide fractions and analyzed as pools. This article describes parallel experiments involving both approaches, designed to take advantage of the StrOligo algorithm functionalities with the aim of characterizing glycosylation microheterogeneity on a specific site. A hybrid quadrupole‐quadrupole‐time‐of‐flight (QqTOF) instrument equipped with a matrix‐assisted laser desorption/ionization (MALDI) source was used. Glycosylation of α5β1 subunits of human integrin was studied to test the methodology. The sample was divided in two aliquots, and glycans from the first aliquot were released enzymatically, labelled with 2‐aminobenzamide, and identified using tandem mass spectrometry (MS/MS) and the StrOligo program. The other aliquot was digested with trypsin and the resulting peptides separated by reversed‐phase high‐performance liquid chromatography (HPLC). A specific collected fraction was then analyzed by MS before and after glycan release. These spectra allowed, by comparison, detection of a glycopeptide (several glycoforms) and elucidation of peptide sequence. Compositions of glycans present were proposed, and identification of possible glycan structures was conducted using MS/MS and StrOligo. Copyright © 2005 John Wiley & Sons, Ltd.
Increasing fat in the diet does not improve muscle performance in patients with mitochondrial myopathy due to complex I deficiency
Authors: de Meer K. , Roef M. J. , de Klerk J. B. C. , Bakker H. D. , Smit G. P. A. , Poll‐The B. T. 
Published: February 2005

SummaryFour myopathic patients with complex I deficiency followed diets containing 55 energy per cent (En%) as fat or 25 En% as fat, both for three weeks. Maximal workload and muscle force were not different on either diet. Exercise endurance time, oxygen consumption and lactate levels were also not different, but one patient had diminished endurance time on 25 En% as fat. Our observations do not support the use of increasing the fat in the diet of patients with mitochondrial complex I deficiency.
Monitoring enzyme replacement therapy in Fabry disease—Role of urine globotriaosylceramide
Authors: Whitfield P. D. , Calvin J. , Hogg S. , O'Driscoll E. , Halsall D. , Burling K. , Maguire G. , Wright N. , Cox T. M. , Meikle P. J. , Deegan P. B. 
Published: February 2005

SummaryAnderson‐Fabry disease (referred to as Fabry disease) is an X‐linked disorder characterized by a deficiency of the lysosomal enzyme α‐galactosidase A and the subsequent accumulation in various tissues of globotriaosylceramide (Gb3), the main substrate of the defective enzyme. Enzyme replacement therapy (ERT) offers a specific treatment for patients with Fabry disease, though monitoring of treatment is hampered by a lack of surrogate markers of response. In this study, the efficacy of long‐term ERT in six Fabry hemizygotes and two symptomatic heterozygotes has been evaluated. Patients were administered recombinant α‐galactosidase A every 2 weeks for up to a year. The efficacy of ERT was assessed by monitoring symptomatology and renal function. Urinary glycolipid concentration was estimated by a novel tandem mass spectrometric method. Urine glycolipid (Gb3) was elevated at baseline and fell impressively on ERT where patients were hemizygotes and in the absence of renal transplantation. In heterozygotes and in a recipient of a renal allograft, elevations and changes in urine glycolipids were less pronounced. In one patient, after several months of ERT, there was a transient increase in Gb3 concentrations to baseline (pre‐ERT) levels, associated with the presence of antibodies to the recombinant α‐galactosidase A. The marked decline in urine Gb3 on ERT, and its subsequent increase in association with an inhibitory antibody response, suggest that this analyte deserves further investigation as a potential marker of disease severity and response to treatment.
Preimplantation diagnosis of a lysosomal storage disorder by in situ enzymatic activity: ‘Proof of principle’ in acid sphingomyelinase‐deficient mice
Authors: Butler A. , Henderson S. C. , Gordon R. E. , Dagan A. , Gatt S. , Schuchman E. H. 
Published: February 2005

SummaryGenetic diagnosis of preimplantation embryos (PGD) can substantially reduce the chance that at‐risk couples have children afflicted with inherited diseases. However, PGD requires DNA,which is usually obtained from single cells following embryo biopsy. In addition, PGD requires that the genetic defect(s) causing the disorder be known. We have therefore developed an alternative to PGD, which we term preimplantation enzymatic diagnosis (PED). PED has several advantages over PGD, including the facts that it does not require embryo biopsy and that the gene defect(s) causing the disorder need not be known. We have demonstrated ‘proof of principle’ for this approach using embryos obtained from a mouse model (ASMKO mice) of acid sphingomyelinase (ASM)‐deficient Niemann‐Pick disease, an inherited lysosomal storage disorder. For this technique, fluorescently (BODIPY)‐conjugated sphingomyelin was used to detect ASM activity in situ. Wild‐type, preimplantation embryos degraded the substrate following a short ‘pulse‐chase’ period, resulting in markedly reduced fluorescence compared to ASMKO embryos, which retained the fluorescent substrate. Thus, the two embryo types could be easily distinguished by fluorescent microscopy. The fluorescent sphingomyelin was not toxic to the embryos, and the entire procedure could be accomplished within 48 h without embryo biopsy. We suggest that PED may be useful for the preimplantation diagnosis of lysosomal storage disorders, and perhaps other enzymatic defects where similar in situ assay methods are available.
Screening for Menkes disease using the urine HVA/VMA ratio
Authors: Matsuo M. , Tasaki R. , Kodama H. , Hamasaki Y. 
Published: February 2005

SummaryMenkes disease is a disorder of copper transport that results in early death. Early therapy with parenteral copper‐histidine has been shown to markedly improve outcomes. However, early diagnosis is difficult because patients are asymptomatic in early infancy. In Menkes disease, impaired activity of dopamine β‐hydroxylase, a copper‐dependent enzyme, leads to increased urine ratios of homovanillic acid/vanillylmandelic acid (HVA/VMA). Urine HVA/VMA ratios ranged from 4.1 to 69.7 among 15 patients with Menkes disease, whereas only 0.18% of controls had ratios greater than 4.0. Thus, the urine HVA/VMA ratio is a useful screening method for Menkes disease.
Severe hyperhomocysteinaemia and 5‐oxoprolinuria secondary to antiproliferative and antimicrobial drug treatment
Authors: Schwahn B. , Kameda G. , Wessalowski R. , Mayatepek E. 
Published: February 2005

SummaryA hitherto healthy 7‐year‐old girl underwent antiproliferative and antibiotic treatment owing to the diagnosis of T‐cell lymphoma and concomitant bacterial infection. She developed an encephalopathic crisis associated with metabolic acidosis, hyponatraemia and severe hyperhomocysteinaemia and 5‐oxoprolinuria. Laboratory tests normalized completely after recovery. Primary defects in glutathione metabolism could be excluded.
Population genetic structure and history of the long-tailed hake, Macruronus magellanicus, in the SW Atlantic as revealed by mtDNA RFLP analysis
Authors: D'Amato M.E. , Carvalho G.R. 
Published: February 2005

AbstractThe long-tailed hake, Macruronus magellanicus, is widely distributed in the SW Atlantic, and although it is commercially exploited, the extent of genetic structuring remains unclear. The hypothesis of a separate inshore stock as suggested by past biological data in northern Patagonia was tested with RFLP profiles of the mtDNA region ND5/6, in a total of 160 animals from eight locations. Sequence divergence between populations was nil, and all diversity is contained within populations. Despite the lack of geographic structuring in the distribution of haplotypes, the inference of population homogeneity should be taken cautiously owing to the skewed frequency distribution of haplotypes, with one allele accounting for 63% of individuals. A phylogenetic analysis of haplotypes reveals a star-shaped pattern with the presence of two lineages that may represent a population expansion. A rapid turnover of lineages, sweepstake recruitment, rapid expansion, or vulnerability to environmental conditions is not only suggested by the network pattern, but also by the departure from neutrality expectations. However, the role of selection cannot be ruled out until more loci or markers are examined.
Accuracy and precision of radiostereometric analysis in the measurement of three‐dimensional micromotion in a fracture model of the distal radius
Authors: Madanat Rami , Mäkinen Tatu J. , Moritz Niko , Mattila Kimmo T. , Aro Hannu T. 
Published: February 2005

AbstractThe purpose of the current study was to verify the feasibility of radiostereometric analysis (RSA) in monitoring three‐dimensional fracture micromotion in fractures of the distal radius. The experimental set‐up consisted of a simulated model of an extra‐articular Colles' fracture, including metallic beads inserted into the bone on either side of the fracture site. The model was rigidly fixed to high precision micrometer stages allowing controlled translation in three axes and rotation about the longitudinal and transverse axes. The whole construct was placed inside a RSA calibration cage with two perpendicular radiographic film cassettes. Accuracy was calculated as the 95% prediction intervals from the regression analyses between the micromotion measured by RSA and actual displacements measured by micrometers. Precision was determined as the standard deviation of five repeated measurements of a 200 μm displacement or a 0.5° rotation along a specific axis. Translations from 25 μm to 5 mm were measured with an accuracy of ±6μm and translations of 200μm were measured with a precision of 2–6 μm. Rotations ranging from 1/6° to 2° were measured with an accuracy of ±0.073° and rotations of 1/2° were measured with a precision of 0.025°–0.096°. The number of markers and their configuration had greater impact on the accuracy and precision of rotation than on those of translation. Aside from the unknown rate of clinical marker loosening, the current results favor the use of at least four markers in each bone fragment in distal radius fractures. These results suggest a strong rationale for the use of RSA as an objective tool for comparing different treatment modalities and novel bone graft substitutes aimed at stabilization of fractures of the distal radius. © 2004 Orthopaedic Research Society. Published by Elsevier Ltd. All rights reserved.