bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical and molecular characterization of two adults with autosomal recessive Robinow syndrome
Authors: Tufan Fatih , Cefle Kivanc , Türkmen Seval , Türkmen Aydin , Zorba Unal , Dursun Memduh , Oztürk Sükrü , Palandüz Sükrü , Ecder Tevfik , Mundlos Stefan , Horn Denise 
Published: June 2005

AbstractAutosomal recessive Robinow syndrome is caused by mutations in ROR2 and is characterized by short stature, mesomelic limb shortening, brachydactyly, vertebral abnormalities, and a characteristic “fetal face” dysmorphology. We report the clinical and molecular studies on two adults with this condition. Besides typical skeletal and facial features, one patient developed hydronephrosis, nephrocalcinosis, and renal failure. The second patient had characteristic skeletal manifestations including severe spinal involvement and showed endocrinological abnormalities including elevated gonadotropic hormones. The facial phenotype in both patients remained distinctive into adulthood. Analysis of the ROR2 gene revealed a homozygous c.1937_1943delACAAGCT mutation in Patient 1, and compound heterozygosity for c.355C > T (p.R119X). and c.550C > T (p.R184C) in Patient 2. © 2005 Wiley‐Liss, Inc.
Effizienz in der nichtenzymatischen kinetischen Racematspaltung
Authors: Vedejs Edwin , Jure Mara 
Published: June 2005

AbstractDas im Vortitel gezeigte Walden‐Denkmal auf dem Campus der Technischen Universität Riga ist sicher eines der wenigen öffentlichen Monumente, die Schlüsselereignisse aus der Anfangszeit der stereokontrollierten Synthese thematisieren und vielleicht das einzige, das die “molekulare Sprache” der organischen Chemie gebraucht. Die ersten nichtenzymatischen kinetischen Racematspaltungen wurden, ebenso wie die ursprünglichen Varianten der Walden‐Umkehr, in den 1890er Jahren untersucht – also noch in der ersten Forschergeneration nach Entdeckung der tetraedrischen Natur des Kohlenstoffs. Aus diesen frühen Arbeiten ist abzulesen, dass die Prinzipien und die Bedeutung enantiokontrollierter Synthesen bereits gut verstanden waren. Was noch fehlte war eine verlässliche und schnelle Methode zur Quantifizierung der Ergebnisse und zur Optimierung der Experimente. Viele Jahrzehnte sollten vergehen, bis das Problem mit Einführung von HPLC‐ und GPLC‐Testreihen auf chiralen Trägern gelöst wurde. Das Walden‐Denkmal lädt den Vorübergehenden zur spielerischen Betrachtung ein – auf ähnliche Weise, wie auch die kinetische Racematspaltung zum Experimentieren einlädt. Dieser Aufsatz verfolgt den Verlauf der Geschichte angefangen von den ersten Entdeckungen über die faszinierenden historischen Meilensteine und konzeptionellen Entwicklungen und schließt mit einem Blick auf moderne Techniken zur Effizienzmaximierung.
Efficiency in Nonenzymatic Kinetic Resolution
Authors: Vedejs Edwin , Jure Mara 
Published: June 2005

AbstractThe Walden memorial at the Technical University in Riga is pictured in the frontispiece to mark the recent centennial of the Walden inversion. This is a rare public monument to key events from the first era of exploration in stereocontrolled synthesis, and may be the only such monument to use the language of organic chemistry expressed at the molecular level. The reaction of racemic substrates with chiral nucleophiles is one of many methods currently known to achieve kinetic resolution, a phenomenon that ranks as the oldest and most general approach for the synthesis of highly enantioenriched substances. The first nonenzymatic kinetic resolutions as well as the original forms of the Walden inversion were studied in the 1890s. All of these investigations were conducted within the first generation following the demonstration that carbon is tetrahedral, and provided abundant evidence that the principles and importance of enantiocontrolled syntheses were understood. However, a reliable, rapid technique to quantify results and guide the optimization process was still lacking. Many decades passed before this problem was solved by the advent of HPLC and GLPC assays on chiral supports, which stimulated explosive growth in the synthesis of nonracemic substances by kinetic resolution. The Walden monument is accessible to passers‐by for hands‐on inspection as well as for contemplation and learning. In a similar way, kinetic resolution is experimentally accessible and can be thought‐provoking at several levels. We follow the story of kinetic resolution from the early discoveries through fascinating historical milestones and conceptual developments, and close with a focus on modern techniques that maximize efficiency.
Preparation and characterization of double‐MF shell microPCMs used in building materials
Authors: Su Jun‐Feng , Wang Li‐Xin , Ren Li 
Published: June 2005

AbstractA kind of double‐shell heat energy storage microcapsule was prepared used melamine formaldehyde (MF) resin as shell material, and the properties of the microcapsules were investigated. A phase change material, with melt point of 24°C and phase transition heat of 225.5J/g, was used as core. The microcapsules would be used in indoor walls to regulate the temperature and save energy. The surface morphological structure was examined by means of scanning electron microscopy. The strength of the shell was evaluated through observing the surface change after pressure by means of scanning electron microscopy. The average diameter of the microcapsules was 5 μm ∼ 10 μm. Diameter of 1 μm ∼ 5 μm could also be obtained by using different stirring speeds. The globular surface was smooth and compact. The thickness was 0.5 μm ∼ 1 μm. Also, the melting point of the microcapsules was 24.7°C, nearly equal to the pure phase change material. The DSC results make clear that the polymer shell of the microcapsules does not influence the properties of the phase change material. It was also found that the avoiding penetration property of the double‐shell microcapsules was better than that of single shell, and the average diameter of 5 μm was better than 1 μm. With the increase of ratio of the core material, the compactability decreased, and the shell thickness decreased. The mass ratio of core and shell was 3 : 1 to ensure that the microcapsules had good heat storage function. The measuring test showed that the microcapsules did not rupture at a pressure of 1.96 × 105 Pa. © 2005 Wiley Periodicals, Inc. J Appl Polym Sci 97: 1755–1762, 2005
Miscibility and fracture toughness of thermoplastic polyurethane/poly(vinyl chloride)/nitrile rubber ternary blends with special reference to the blend composition
Authors: Manoj N. R. , Sivaraman P. , Praveen S. , Raut R. D. , Chandrasekhar L. , Chakraborty B. C. 
Published: June 2005

AbstractTernary blends of thermoplastic polyurethane and a poly(vinyl chloride)/nitrile rubber blend were investigated in this work. The blends, with weight ratios of 100/0, 80/20, 40/60, 60/40, 80/20, and 0/100, were prepared via melt blending. Dynamic mechanical analysis showed that the blends with ratios of 20/80 and 80/20 were miscible, whereas the 40/60 and 60/40 blends were partially miscible. IR spectroscopy studies showed shifts in the peaks due to specific interactions in the blends. The blends showed degradation behavior between the blend components. The fracture toughness was investigated with the J‐integral by the locus method; the components and the miscible blends had good fracture toughness, whereas the other blends had lower toughness. Similar behavior was observed for the tensile properties. Scanning electron microscopy studies showed the morphological variations in the blends. © 2005 Wiley Periodicals, Inc. J Appl Polym Sci 97: 1763–1770, 2005
Mechanical properties of starch‐based materials. I. Short review and complementary experimental analysis
Authors: Follain N. , Joly C. , Dole P. , Bliard C. 
Published: June 2005

AbstractThe aim of this study was to review the mechanical properties (at break) of plasticized starch‐based materials from the literature. The methodology relied on the use of a graphic tool allowing a direct comparison of the strength and strain at break. The mechanical properties of the materials were systematically compared with those of glycerol‐plasticized starches at 57% relative humidity. Because all the systems were equivalent, whatever the strategies were, several starch development approaches were examined that depended on the required performance at break. First, classical formulations described in the literature (under comparable conditions) were tested. A smoothing of the mechanical performances was obtained, and they were surprisingly similar to those of the simple glycerol‐plasticized, starch‐based material family. © 2005 Wiley Periodicals, Inc. J Appl Polym Sci 97: 1783–1794, 2005
Anisotropic network formation by the photopolymerization of new monomers derived from 4‐hydroxybenzenethiol
Authors: Sáez P. , Aguilera C. 
Published: June 2005

AbstractNew polymerizable difunctional liquid crystals of monomers derived from 4‐hydroxybenzenethiol were synthesized, characterized, and photopolymerized by the formation of anisotropic films. These films were obtained by the irradiation of the monomers in the mesophase with UV light before they were macroscopically oriented in glasses treated on the surface with polyimide and uniaxially rubbed. The monomers showed smectic and nematic mesophases. The thin films, uniaxially oriented, were optically transparent. The orientation was verified by IR dichroism, which showed a preferential order of mesogens. © 2005 Wiley Periodicals, Inc. J Appl Polym Sci 97: 1812–1817, 2005
Effects of hydroxyl groups and architecture of organic polymers on polystyrene/silica nanocomposites
Authors: Saito Reiko , Kobayashi Shin‐Ichiro , Hosoya Takayoshi 
Published: June 2005

AbstractTransparent polystyrene/silica nanocomposites were synthesized by casting the blend solutions of polystyrene (PS) derivatives and perhydropolysilazane (PHPS) and by calcinating the blend films of PS derivatives and PHPS at 100°C for 3 h under steam. Poly(styrene‐co‐4‐vinyl phenol)s [P(S‐co‐VPh)s], poly(styrene‐co‐hydroxymethyl styrene)s [P(S‐co‐HMS)s], poly(styrene‐co‐4‐vinyl phenol)‐block‐PSs [P(S‐co‐VPh)‐block‐PSs], and poly(styrene‐co‐hydroxymethyl styrene)‐block‐PSs [P(S‐co‐HMS)‐block‐PSs] were used as the PS derivatives. The morphology of the microphase separation, transparency, the degree of grafting of PHPS onto PS derivatives, and surface hardness of the composites were investigated by transmission electron microscopy, UV–vis, FTIR, and nanoindentation, respectively. Degrees of grafting of PHPS onto PS derivatives and transparency of the composites of P(S‐co‐VPh)s and P(S‐co‐VPh)‐block‐PSs were higher than those of P(S‐co‐HMS)s and P(S‐co‐HMS)‐block‐PSs. Surface hardness of the composites of P(S‐co‐VPh)‐block‐PS was drastically increased from 0.22 to 0.55 GPa by increasing the weight fraction of silica to 33.7%. © 2005 Wiley Periodicals, Inc. J Appl Polym Sci 97: 1835–1847, 2005
Hypertonic upregulation of betaine transport in renal cells is blocked by a proteasome inhibitor
Authors: Lammers Philip E. , Beck Jeffrey A. , Chu Shaoyou , Kempson Stephen A. 
Published: June 2005

AbstractThe renal betaine transporter (BGT1) protects cells in the hypertonic medulla by mediating uptake and accumulation of the osmolyte betaine. Transcription plays an essential role in upregulating BGT1 transport in MDCK cells subjected to hypertonic stress. During hypertonic stress, the abundance of the transcription factor TonEBP increases and it shifts from the cytoplasm to the nucleus where it activates transcription of the BGT1 gene. Little is known about post‐transcriptional regulation of BGT1 protein. In the presence of the proteasome inhibitor MG‐132, which blocked nuclear translocation of TonEBP, the hypertonic upregulation of BGT1 protein and transport was prevented and cell viability in hypertonic medium was impaired over 24 h. Urea also prevented the hypertonic upregulation of BGT1 protein and transport, but did not interfere with TonEBP translocation and cell viability. Shorter treatments of hypertonic cells with MG‐132 avoided viability problems and produced dose‐dependent inhibition of translocation and transport. When stably transfected MDCK cells that over‐expressed BGT1 were treated for 6 h with hypertonic medium containing 3 µM MG‐132, there was 43% inhibition of nuclear translocation, 83% inhibition of BGT1 transport, and no change in viability. While other proteasome functions may be involved, these data are consistent with a critical role for nuclear translocation of TonEBP in upregulation and membrane insertion of BGT1 protein. Copyright © 2005 John Wiley & Sons, Ltd.
Typhlitis in childhood cancer
Authors: McCarville M. Beth , Adelman C. Scott , Li Chenghong , Xiong Xiaoping , Furman Wayne L. , Razzouk Bassem I. , Pui Ching‐Hon , Sandlund John T. 
Published: June 2005

AbstractBACKGROUNDTyphlitis is increasingly recognized in children undergoing chemotherapy but is poorly characterized. The authors investigated the demographic, clinical, and imaging (ultrasonography and computed tomography [CT] scans) variables related to the diagnosis, risk, and outcome of typhlitis.METHODSThe authors reviewed the records of patients who had typhlitis (bowel wall thickness ≥ 0.3 cm plus clinical findings) during treatment at St. Jude Children's Research Hospital (Memphis, TN) between 1990 and 2001. They assessed whether duration of typhlitis was related to bowel wall thickness, extent of colonic involvement, ascites, demographics, primary diagnosis, symptoms of typhlitis, or duration of neutropenia. To identify risk factors for typhlitis, the authors compared the demographic data and previous drug therapy of 78 patients who had typhlitis and 1231 identically treated children who did not.RESULTSOf 3171 children, 83 (2.6%) developed typhlitis. Frequent symptoms were abdominal pain (91%), fever (84%), abdominal tenderness (82%), and diarrhea (72%). Twelve percent of the patients were not neutropenic. Duration of typhlitis was associated with bowel wall thickness measured by ultrasonography (n = 68; P = 0.05) but not CT scan (n = 48; P = 0.67) and was associated with duration of neutropenia (P = 0.02), fever (P = 0.01), and abdominal tenderness (P = 0.04). Age >16 years at cancer diagnosis was the only demographic factor associated with typhlitis (P = 0.03). Two patients died of typhlitis.CONCLUSIONSUltrasonography was a useful imaging modality for children with suspected typhlitis. The classic triad of abdominal pain, fever, and neutropenia may be absent. The severity of typhlitis was related to the duration of neutropenia and the presence of fever or abdominal tenderness. Cancer 2005. © 2005 American Cancer Society.
Serum HER‐2/neu conversion to positive at the time of disease progression in patients with breast carcinoma on hormone therapy
Authors: Lipton Allan , Leitzel Kim , Ali Suhail M. , Demers Laurence , Harvey Harold A. , Chaudri‐Ross Hilary A. , Evans Dean , Lang Raquel , Hackl Wolfgang , Hamer Peter , Carney Walter 
Published: June 2005

AbstractBACKGROUNDProlonged exposure of breast carcinoma cells in vitro to tamoxifen results in tamoxifen resistance. Tamoxifen‐resistant cells express increased HER‐2/neu mRNA and protein. The objective of this study was to determine whether patients with metastatic or locally advanced breast carcinoma who have negative serum HER‐2/neu status at the initiation of first‐line hormone therapy with letrozole or tamoxifen convert to positive serum HER‐2/neu status at the time of disease progression and to determine whether serum HER‐2/neu conversion to positive status is associated with response to therapy and overall survival.METHODSSerum samples were obtained at baseline and at the time of disease progression from 240 patients who initially had negative serum HER‐2/neu status (< 15 ng/mL). A manual microtiter, enzyme‐linked immunosorbent assay that was specific for the extracellular domain of the HER‐2/neu (c‐erbB‐2) oncoprotein product was used to quantitate serum levels.RESULTSAmong 240 patients, 61 patients (26%) converted from serum HER‐2/neu negative to positive (> 15 ng/mL) at the time of disease progression. Thirty‐two of 129 patients (25%) who were treated with tamoxifen and 29 of 111 patients (26%) who were treated with letrozole became converted to positive serum HER‐2/neu status at the time of disease progression. The response rate and the time to disease progression on first‐line hormone therapy were not affected by serum HER‐2/neu conversion. The survival of patients who converted to positive serum HER‐2/neu status was significantly shorter compared with the survival of patients who remained negative for serum HER‐2/neu. A multivariate analysis revealed that conversion to positive serum HER‐2/neu status was an independent prognostic variable for survival.CONCLUSIONSConversion to positive serum HER‐2/neu status occurred in approximately 25% of patients who received first‐line hormone therapy. Conversion to serum HER‐2/neu‐positive status occurred with equal frequency in antiestrogen and aromatase‐inhibitor therapy. The current results showed that serum conversion to HER‐2/neu‐positive status was an independent risk factor for decreased survival in patients with breast carcinoma. Cancer 2005. © 2005 American Cancer Society.
Finite element methods for the simulation of incompressible powder flow
Authors: Ouazzi A. , Turek S. , Hron J. 
Published: June 2005

AbstractUnlike fluids, flowing powders do not exhibit viscosity such that a Newtonian rheology cannot accurately describe granular flow. Assuming that the material is incompressible, dry, cohesionless, and perfectly rigid‐plastic, and based on continuum theories, generalized Navier–Stokes equations (‘Schaeffer Model’) have been derived where the velocity gradient has been replaced by the shear rate, and the viscosity depends on pressure and shear rate which leads to mathematically complex problems. In this note we present numerical algorithms to approximate these highly non‐linear equations based on finite element methods. A Newton linearization technique is applied directly to the corresponding continuous variational formulations. The approximation of incompressible velocity field is treated by using stabilized non‐conforming Stokes elements and we use a pressure Schur complement smoother as defect correction in a direct multigrid approach to solve the linear saddle‐point problems with high numerical efficiency. The results of several computational experiments for prototypical flow configurations are provided. Copyright © 2005 John Wiley & Sons, Ltd.
How Good is Fluorine as a Hydrogen‐Bond Acceptor in Fluorinated Single‐Walled Carbon Nanotubes?
Authors: Bettinger Holger F. 
Published: June 2005

AbstractAb initio (RI‐MP2/TZVPP) computations were employed to investigate the interaction between hydrogen‐bond donors H2O and CH3OH and covalently bound fluorine in organofluorine compounds. While the CF⋅⋅⋅HO interaction energy is around 3 kcal mol−1 for unstrained systems, the linear correlation between pyramidalization angle at the carbon atom and the interaction energy suggests that increased binding can be obtained in strained systems. This is confirmed for the dihydrodifluoropyrene–methanol pair, but a large portion of the binding energy is due to the interaction of the π system with the oxygen atom. Density functional periodic boundary condition computations (PBC‐PBE/6‐31G*) of the structures of (5,5) and (10,10) armchair (C2F)n fluorinated SWNTs (F‐SWNTs) indicate that the pyramidalization at the fluorine‐binding carbon atoms are too similar to that of CH3F to enhance the hydrogen‐bond acceptor properties of fluorine significantly. The solubility of F‐SWNTs in alcohols therefore could be due to a combination of hydrogen bonds and van der Waals interactions with the π systems.
Interfacial Langmuir–Blodgett Assembly of Straight and Parallel Aligned Nanoribbons
Authors: Chen Penglei , Gao Peng , Zhan Chuanlang , Liu Minghua 
Published: June 2005

AbstractThe fabrication of one‐dimensional nanomaterials is currently an important issue in nanoscience and nanotechnology. Herein, we report the construction of long, straight, and parallel aligned nanoribbons, by the in situ assembly of a novel, triangular amphiphilic molecule at an air–water interface. The 1,3,5‐trimesyl‐tri‐L‐glutamic acid hexaester, which has an aromatic core with three hydrophilic arms bearing three amide units and ending with six ester groups, was spread on a water surface, forming a stable, ultrathin film with a plateau region in its isotherm. When the film was compressed to a surface pressures, which exceeded the plateau region, nanoribbons were formed. Atomic force microscopy, X‐ray diffraction, Fourier‐transform infrared spectroscopy, transmission electron microscopy, electron diffraction, and circular dichroism were used to characterize the film and the nanoribbons. The nanoribbons were formed due to the in situ transformation of a two‐dimensional monolayer into a three‐dimensional nanoarchitecture at the air–water interface. When the compound was spread onto the water surface, it first formed a monolayer. Compressing the film led to the overlap of the second layer on the monomolecular sublayer. This second layer formed nanoribbons in which the triangular molecules stood vertically (using two corners) on the sublayer. The nanoribbons were stabilized by the ordered packing of the triangular molecules, which were held together by intermolecular hydrogen bonding. The results present a facile procedure for constructing three‐dimensional, regular nanoarchitectures in a two‐dimensional environment.
Angle‐Resolved Photoelectron Spectroscopy of Randomly Oriented 3‐Hydroxytetrahydrofuran Enantiomers
Authors: Giardini Anna , Catone Daniele , Stranges Stefano , Satta Mauro , Tacconi Mario , Piccirillo Susanna , Turchini Stefano , Zema Nicola , Contini Giorgio , Prosperi Tommaso , Decleva Pietro , Di Tommaso Devis , Fronzoni Giovanna , Stener Mauro , Filippi Antonello , Speranza Maurizio 
Published: June 2005

AbstractCircular dichroism in the angular distribution of valence photoelectrons emitted from randomly oriented 3‐hydroxytetrahydrofuran enantiomers (ThS and ThR) has been observed in gas‐phase experiments using circularly polarized vacuum ultraviolet (VUV) light. The measured dichroism for both ThS and ThR, acquired at the single magic angle θ=234.73 ° and at photon energies of 22, 19, 16, and 14 eV, points to an asymmetric forward–backward scattering of the photoelectrons from their highest occupied molecular orbitals (HOMO) HOMO‐1 and HOMO‐2, of up to 5 %, depending on the photon energy. The asymmetry reverses on exchange of either the helicity of the radiation or the configuration of Th. The photoionization dichroic D parameters of ThS and ThR have been measured and their values discussed in the light of LCAO B‐spline density functional theory (DFT) predictions. While an acceptable agreement is found between the dichroic parameter measured and calculated at the highest photon energy for the HOMO and HOMO‐2 orbitals of Th, a significant discrepancy is observed for the HOMO‐1 state which is attributed to the floppiness of Th, in particular to the comparatively large sensitivity of the size and shape of its HOMO‐1 on nuclear vibrational motion.