bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical and molecular studies on two further families with Simpson‐Golabi‐Behmel syndrome
Authors: Rodríguez‐Criado Germán , Magano Luis , Segovia Mabel , Gurrieri Fiorella , Neri Giovanni , González‐Meneses Antonio , Gómez de Terreros Ignacio , Valdéz Rita , Gracia Ricardo , Lapunzina Pablo 
Published: September 2005

AbstractThe Simpson‐Golabi‐Behmel syndrome (SGBS) (OMIM 312870) is an overgrowth/multiple congenital anomalies syndrome caused by a semi‐dominant X‐linked gene encoding glypican 3 (GPC3). It shows great clinical variability, ranging from mild forms in carrier females to lethal forms with failure to thrive in males. The most consistent findings in SGBS are pre‐ and postnatal macrosomia, characteristic facial anomalies and abnormalities affecting the internal organs, skeleton, and on some occasions, mental retardation of variable degree. SGBS is also associated with an increased risk of developing embryonal tumors, mostly Wilms and liver tumors. We describe two molecularly‐confirmed families with SGBS. All patients had typical manifestations of SGBS including some female relatives who had minor manifestations of the disorder. Some patients had novel findings such as a deep V‐shaped sella turcica and six lumbar vertebrae. Molecular studies in affected patients showed a deletion of exon 6 in family 1 and an intronic mutation in family 2. © 2005 Wiley‐Liss, Inc.
Chromosome 11‐q24 region in Tourette syndrome: Association and linkage disequilibrium study in the French Canadian population
Authors: Díaz‐Anzaldúa Adriana , Rivière Jean‐Baptiste , Dubé Marie‐Pierre , Joober Ridha , Saint‐Onge Judith , Dion Yves , Lespérance Paul , Richer Francois , Chouinard Sylvain , Rouleau Guy Armand ,   The Montreal Tourette Syndrome Study Group
Published: September 2005

AbstractPrevious studies have found association and linkage between Tourette syndrome (TS) and markers at the 11q24 region, mainly with markers D11S1377 and D11S933. In order to determine if these positive findings could be replicated in our sample, we undertook a family‐based association study in 199 French Canadian TS nuclear families. We genotyped 572 individuals from 174 complete and 25 incomplete TS trios. TDT analysis failed to detect an association between TS and six markers from 11q24. Furthermore, no haplotype combining alleles from D11S1377, D11S933, or any of the other four markers was associated with the disorder. Linkage disequilibrium analysis showed evidence of historical recombination between every contiguous pair of markers, indicating that these genetic variants are probably in equilibrium in the French Canadian population. Further analysis in additional families, with different methodologies (linkage and association) will be required in order to determine if the 11q24 region harbors a susceptibility locus for TS. If it does, this defect may not be frequent in the French Canadian population due to locus heterogeneity. © 2005 Wiley‐Liss, Inc.
New Approach to the ALD of Bismuth Silicates; Bi(CH2SiMe3)3 Acting as a Precursor for both Bismuth and Silicon
Authors: Harjuoja J. , Hatanpää T. , Vehkamäki M. , Väyrynen S. , Putkonen M. , Niinistö L. , Ritala M. , Leskelä M. , Rauhala E. 
Published: September 2005

AbstractBismuth silicate thin films were deposited using atomic layer deposition (ALD) with a novel precursor, Bi(CH2SiMe3)3, serving as both bismuth and silicon source. Precursor synthesis, analysis, and crystal structure are also reported. Bi(CH2SiMe3)3 forms hexagonal crystals with a = 10.7110(11) Å, b = 10.7110(11) Å, c = 10.2500(7) Å; space group P63. The deposition temperature of thin films was 200–450 °C, where a constant growth rate of 0.4 Å per cycle was obtained between 250 °C and 350 °C. Impurity levels of bismuth silicate films deposited at 250 °C were below 0.2 at.‐ % and 0.1 at.‐ % for carbon and hydrogen, respectively. The as‐deposited films were amorphous, and post‐synthetic annealing in an atmosphere of N2 or O2 at 400–1000 °C was applied.
Deposition of Palladium from a Cylcopentadienyl‐allyl‐palladium Precursor on Si‐Based Substrates with Various Pretreatments: The Role of Surface Si–OH and Si‐H Species Studied by X‐Ray Photoelectron Spectroscopy
Authors: Wu Q.‐H. , Gunia M. , Strunskus T. , Witte G. , Muhler M. , Wöll C. 
Published: September 2005

AbstractThe decomposition reactions of a metal‐organic precursor for the CVD of Pd, (Cp)Pd(allyl), on Si substrates with various terminated surfaces have been studied using X‐ray photoelectron spectroscopy (XPS). The XPS data show that mixed hydrogen/hydroxyl‐terminated Si surfaces exhibit the highest activity ascribed to acidic Si‐OH groups for precursor decomposition, followed by the as‐received Si surface. Clean, well‐defined SiO2 surfaces prepared in ultrahigh vacuum (UHV) exhibit the lowest activity for the Pd precursor decomposition, whereas the hydrogen‐terminated Si surface was rather active. Scanning electron microscopy (SEM) images show that Pd clusters were formed on the mixed hydrogen/hydroxyl‐terminated Si surface with a coverage of about 10 %. The average size of the Pd clusters is about 30 nm. Based on the XPS experimental data, a hydrogen‐assisted decomposition mechanism of the Pd precursor is proposed.
Continuous Synthesis of Y‐Junction Carbon Nanotubes by Catalytic CVD
Authors: Su L. F. , Wang J. N. , Yu F. , Sheng Z. M. 
Published: September 2005

AbstractCupric acetate dissolved in methanol was applied, for the first time, to the synthesizing of Y‐junction carbon nanotubes (CNTs). This was done by catalytic CVD of the methanol solution at 1000–1200 °C. Based on the bamboo structure, which was observed for the first time within Y‐junction CNTs, it is suggested that the growth of such CNTs may be a result of the fluctuation of external conditions causing a change in the growth direction of CNTs developed from completely melted Cu nanoparticles. Since the production process is continuous, the present technique can easily be scaled up for large‐scale production for a wide variety of applications.
Cationic Silver Coordination Compounds of Polydentate Ligands: Supramolecular Structures of [Ag(Pzbp2Py)2(OSO2CF3)] and [Ag2(Pzbp2Py)2(OSO2CF3)2] {Pzbp2Py = 2‐[3,5‐Bis(4‐butoxyphenyl)pyrazol‐1‐yl]pyridine}
Authors: Gallego M. Luz , Cano Mercedes , Campo José A. , Heras José V. , Pinilla Elena , Torres M. Rosario , Cornago Pilar , Claramunt Rosa M. 
Published: September 2005

AbstractThe new silver complexes [Ag(Pzbp2Py)2(OSO2CF3)], [Ag2(Pzbp2Py)2(OSO2CF3)2], [Ag(PzbpPy)2(OSO2CF3)], [Ag3(TPzbp2Tz)(OSO2CF3)3] and [Ag3(TPzbpTz)(OSO2CF3)3] (5–9) have been obtained by reaction of Ag(OSO2CF3) with the corresponding polydentate ligand 2‐[3,5‐bis(4‐butoxyphenyl)pyrazol‐1‐yl]pyridine (Pzbp2Py, 1), 2‐[3‐(4‐butoxyphenyl)pyrazol‐1‐yl]pyridine (PzbpPy, 2), 2,4,6‐tris[3,5‐bis(4‐butoxyphenyl)pyrazol‐1‐yl]‐1,3,5‐triazine (TPzbp2Tz, 3) and 2,4,6‐tris[3‐(4‐butoxyphenyl)pyrazol‐1‐yl]‐1,3,5‐triazine (TPzbpTz, 4), respectively. The coordination effects induced on the 1H, 13C and 15N NMR chemical shifts of the complexes in solution and in the solid state have been quantified, with the 15N NMR spectra being used as a tool to establish the coordination site. All complexes are consistent with an N,N′‐chelating coordination at each silver centre involving nitrogen atoms from pyrazole and pyridine groups for 5 and 7 or from pyrazole and triazine groups for 8 and 9. An additional Ag···OSO2CF3 interaction completes the environment around the silver atom, giving rise to a five‐coordination or a three‐coordination for complexes 5 and 7 and 8 and 9, respectively. The X‐ray structures of 5 and 6 confirm the presence of the N,N′‐chelating coordination of the Pzbp2Py ligands and the coordinative participation of the CF3SO3 counterion in the metal environment. The Ag···η2‐arene bonds between neighbouring units in 6 are responsible for the dimers containing four‐coordinate silver atoms. At a supramolecular level, Ag···O, C–H···O or C–H···F interactions generate a 2D network in both compounds. (© Wiley‐VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2005)
Metal Binding Within a Peptide‐Based Nucleobase Stack with Tuneable Double‐Strand Topology
Authors: Küsel Andrea , Zhang Jinhua , Alvariño Gil Miguel , Stückl A. Claudia , Meyer‐Klaucke Wolfram , Meyer Franc , Diederichsen Ulf 
Published: September 2005

AbstractPeptide nucleic acid (PNA) oligomers with linear topology are synthesised with histidines at the central positions in order to provide metal‐ion coordination sites within the water shielded and non‐polar environment of a nucleobase stack that emerges upon duplex formation. Variation of the linker length used for attachment of the nucleobases to the regular peptide backbone allows for fine tuning of the distances and coordination environment at the metal binding site. The effect of zinc and copper incorporation into the modified alanyl‐PNA scaffolds on duplex stability was probed by temperature‐dependent UV spectroscopy, and additional insight was gained from EPR spectroscopy and EXAFS. Whenever four histidines (two per oligomer strand) are available, addition of Zn2+ significantly enhances double‐strand stability, thus supporting coordination of Zn2+ by the histidine‐N and incorporation of the metal ion within the base stack. In contrast to Zn2+, however, Cu2+ ions do not induce double‐strand formation of an alanyl/norvalyl‐PNA oligomer, as confirmed by EPR and EXAFS, which reveal a mixed {N2Ox} donor environment. While metal‐ion binding to histidine‐modified alanyl‐PNA oligomers (and homologues) is apparently feasible, a proper matching of the binding‐site geometry and the stereoelectronic requirements of the specific metal ion are essential in order to mediate double‐strand formation. This might offer new options for alternative pairing schemes in alanyl‐PNA chemistry in which metal coordination substitutes the common base‐pair H‐bonding pattern, and for metal‐dependent regulation of alanyl‐PNA duplex formation. (© Wiley‐VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2005)
Shape‐Induced Enhanced Luminescent Properties of Red Phosphors: Sr2MgSi2O7:Eu3+ Nanotubes
Authors: Wan Junxi , Wang Zhenghua , Chen Xiangying , Mu Li , Qian Yitai 
Published: September 2005

AbstractSr2MgSi2O7:Eu3+ nanotubes with red phosphor luminescent properties were successfully synthesized via a low‐temperature hydrothermal route in order to understand the underlying relationship between shape and luminescent properties. The one‐dimensional shape results in an obvious improvement of color purity. From emission spectra, the relative intensity of 5D0→7F2 to 5D0→7F1 transitions in tubular phosphors is higher than that of bulk material. The better color purity results from the more distorted lattices and relatively lower crystal symmetry around Eu3+ ions, which ascribes to the large surface area due to synthesize it in the form of nanotubes. Furthermore, the as‐synthesized tubular red phosphor has shorter fluorescent lifetime and blue‐shiftphenomenon in excitation spectra than that of the bulk counterpart. Based on these results, shape‐induced luminescent properties improvement is proposed as an efficient way to obtain red phosphors with high color purity. (© Wiley‐VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2005)
A New Model of Light‐Powered Chiral Molecular Motor with Higher Speed of Rotation, Part 1 – Synthesis and Absolute Stereostructure
Authors: Fujita Takuma , Kuwahara Shunsuke , Harada Nobuyuki 
Published: September 2005

AbstractTo develop a molecular motor with a higher speed of rotation, a new model light‐powered chiral molecular motor 2 of five‐membered ring type was designed, and the motor rotation isomers (–)‐2a and (–)‐2c were directly synthesized in enantiopure forms for the first time from the ketone (+)‐8. The precursor alcohols 9 and 10 were enantioresolved by the camphorsultam‐dichlorophthalic acid (CSDP acid; 3) method, and their absolute configurations were determined by X‐ray crystallographic analysis of the CSDP ester (–)‐11b and chemical correlations. The enantiopure ketone (S)‐(+)‐8 formed from (1S,2S)‐(+)‐9 or (1R,2S)‐(–)‐10 was subjected to the McMurry reaction with TiCl3/LiAlH4, yielding the motor rotation isomers [CD(–)257.8]‐(2S,2′S)‐(M,M)‐(E)‐(–)‐2a and [CD(–)270.0]‐(2S,2′S)‐(M,M)‐(Z)‐(–)‐2c. These studies enabled us unambiguously to determine the absolute stereostructure of the motor 2, which is of critical importance for control over the direction of motor rotation. (© Wiley‐VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2005)
A New Model of Light‐Powered Chiral Molecular Motor with Higher Speed of Rotation, Part 2 – Dynamics of Motor Rotation
Authors: Kuwahara Shunsuke , Fujita Takuma , Harada Nobuyuki 
Published: September 2005

AbstractTo clarify the mechanism and dynamics of the new model of light‐powered chiral molecular motor (2) of five‐membered ring type, the second motor rotation isomer, the unstable cis‐olefin 2b, was isolated and its stereostructure was determined. Similarly, the fourth motor rotation isomer, the unstable trans‐olefin 2d, was detected by 1H NMR at low temperature, and its stereostructure was also determined. With the aid of this knowledge of the stereostructures of the motor rotation isomers and their spectroscopic data, the dynamics of the motor rotation 2a → 2b → 2c → 2d → 2a were studied by 1H NMR and CD, revealing that the fourth rotation step 2d → 2a was much accelerated, as expected from the design of the new motor. Continuous rotation of this motor was achieved by repetition of the operations of photoirradiation and heating. (© Wiley‐VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2005)
Synthesis and Physicochemical Characterization of meso‐Functionalized Corroles: Precursors of Organic–Inorganic Hybrid Materials
Authors: Barbe Jean‐Michel , Canard Gabriel , Brandès Stéphane , Guilard Roger 
Published: September 2005

AbstractCobalt(III) corroles exhibit an infinite selectivity for the coordination of carbon monoxide towards dioxygen and dinitrogen. This peculiar property thus allows their use as sensing devices for CO detection. Here are described the syntheses and physico‐chemical characterization of meso mono‐, bis‐ and tris(triethoxysilyl)‐functionalized corroles, precursors of organic–inorganic materials. The corrole ring formation was achieved in every case using the “2+1” method involving the reaction of two equivalents of an encumbered dipyrromethane with one equivalent of an aromatic aldehyde in the presence of a catalytic amount of trifluoroacetic acid. The functionalization of the corrole by triethoxysilyl chains was carried out by a condensation reaction of an isocyanate, bearing a triethoxysilyl termination, either on an amino or hydroxy group. Each final compound and intermediate were characterized by various physico‐chemical techniques such as 1H NMR, UV/Vis, MALDI/TOF or EI mass spectrometry and elemental analysis. (© Wiley‐VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2005)
A Convenient Method for the Generation of Allylic Dihaloboranes and Diallyl(chloro)borane and Their Application in the Allylboration of Alkenes and Acetylenes
Authors: Bubnov Yuri N. , Kuznetsov Nikolai Y. , Pastukhov Fedor V. , Kublitsky Vadim V. 
Published: September 2005

AbstractA convenient approach to the highly reactive allylic dihaloboranes and diallyl(chloro)borane based on exchange reactions between BHal3 (Hal = Cl, Br) and allylic triorganoboranes (allyl, cinnamyl and 2‐methylenecyclobutane derivatives) has been developed. The compounds thus generated readily react with terminal alkenes and acetylenes to form the corresponding cis‐1,2‐allylboration products. These products were isolated by standard techniques and transformed into boronates (boron amides). Deboronation of these products led to 1,4‐pentadiene derivatives or unsaturated alcohols. 2‐Substituted 1,4‐pentadienyl(dichloro)boranes obtained from acetylenes underwent intramolecular chloroboration at a moderate temperature to form 5‐chloro‐2‐borinene derivatives. (© Wiley‐VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2005)
Comparison of flame spread of textiles and burn injury prediction with a manikin
Authors: Rossi Rene M. , Bruggmann Georg , Stämpfli Rolf 
Published: September 2005

AbstractThe flame propagation rate of 94 different natural and synthetic fabrics and commercially available garments was assessed using the EN 1103 bench scale test apparatus. To further evaluate the potential burn hazard, the fabrics were then formed into upper garments, put on a full‐scale manikin equipped with 122 heat flux sensors and ignited with a small flame. By using a burn prediction model, the time to feel pain on the human skin as well as the time to suffer second degree burns could be calculated. The bench scale and full‐scale test data measurements show that the flame propagation rate is inversely proportional to the fabric weight for cellulose materials. The fabrics with the highest flame propagation rates were also the garments with the shortest times to pain and to second degree burns on the manikin. However, some blends of natural and synthetic fibres gave short pain and burn times on the manikin, even when the measured flame propagation rate using EN 1103 was low. Therefore, the flame propagation rate is a good means to predict the potential hazard of fabrics made of natural fibres; for synthetics and blends of natural and synthetic fibres, the heat transfer to the skin has to be considered as well. Copyright © 2005 John Wiley & Sons, Ltd.
Fire behaviour of polyester–clay nanocomposites
Authors: Gianelli Walter , Camino Giovanni , Tabuani Daniela , Bortolon Vittorio , Savadori Tonino , Monticelli Orietta 
Published: September 2005

AbstractThe aim of this study was on the one hand, to compare the fire behaviour of polyester/clay nanocomposites with that of the neat polymers added with the conventional flame retardant melamine isocyanurate (MIC) and on the other hand, to study the effect of the flame retardant added to the nanocomposites.Polyester/clay nanocomposites were prepared by using polybutylene terephthalate (PBT) and a co‐polyester elastomer as polymeric matrices and a commercial organoclay as filler.As verified by X‐ray diffractometry (XRD) and transmission electron microscopy (TEM) intercalated structures were obtained by mixing the molten polymer with the layered silicate in a corotating twin screw extruder.The fire behaviour of these materials was investigated by means of an oxygen consumption calorimetry (Cone Calorimeter). Peak heat release rate of 3‐mm thick samples measured at 50 kW/m2 external heat flux was reduced by a factor of 2–3 by the addition of organically modified montmorillonite to the polymers. The further addition of MIC did not give a significant improvement in the behaviour of the materials. Copyright © 2005 John Wiley & Sons, Ltd.
Choroid plexus ependymal cells host neural progenitor cells in the rat
Authors: Itokazu Yutaka , Kitada Masaaki , Dezawa Mari , Mizoguchi Akira , Matsumoto Naoya , Shimizu Akira , Ide Chizuka 
Published: September 2005

AbstractWe previously demonstrated that choroid plexus epithelial (modified ependymal) cells (CPECs) differentiated into astrocytes after grafting into the spinal cord. In the present study, we examined whether CPECs from rats at postnatal 1 day (P1), 7 day (P7), and 8 weeks (P8W) can function as neural progenitor cells that give rise to neurons and glial cells. Cell spheres were produced in cultures of whole tissue of the choroid plexus from the fourth ventricle of rats at each postnatal period. β‐tubulin class III (Tuj‐1), glial fibrillary acid protein (GFAP)‐, and O4‐positive cells differentiated from cell spheres in the differentiation medium. We produced a monoclonal antibody 3E6 specifically labeling microvilli of CPECs. Using this monoclonal antibody, CPECs were isolated from the choroid plexus of P8W rats by cell sorter (FACS). Immunocytochemistry confirmed that there was no contamination from fibroblasts, endothelial cells, macrophages, or Schwann cells in the FACS‐isolated 3E6‐labeled cells. Cell spheres formed in the cultures of these 3E6‐labeled CPECs. After expansion, these cell spheres gave rise to Tuj‐1‐ (5%), GFAP‐ (45%), and O4‐positive cells (0.16%). The remaining cells (45%) were unlabeled neural or glial markers. Some CPECs of the P8W rat were immunohistochemically stained with lineage‐associated markers of Musashi‐1 and epidermal growth factor‐receptor (EGF‐R). In addition, infusion of EGF or fibroblast growth factor‐2 (FGF2) into the ventricle increased the number of bromodeoxyuridine (BrdU)‐positive cells among CPECs from 0.03% (untreated) to 1.14% (38‐fold, EGF) and 1.03% (35‐fold, FGF2), respectively. These findings indicate that neural progenitor cells exist among CPECs in the rat. © 2005 Wiley‐Liss, Inc.
Cortical progenitor cells in the developing human telencephalon
Authors: Howard Brian , Chen Yanhui , Zecevic Nada 
Published: September 2005

AbstractRadial glial (RG) cells have been demonstrated to be a major neural progenitor cell type, but in the human fetal brain, neither their molecular nor their spatiotemporal characteristics are well known. We used glial and neuronal‐specific antibodies to determine the antigen characteristics and distribution of RG cells and other neuronal progenitors in the human brain during the first half of intrauterine development. Proliferating RG (4A4+) cells in the ventricular zone (VZ) showed clear caudorostral and ventrodorsal gradients, spreading from the spinal cord to the ventral rhombencephalon, at embryonic stages (4.5–5.5 gestational weeks [gw]). However, in the same embryo, other dividing cells expressed the neuronal marker SMI‐31 and were present throughout the entire CNS, including the rostral prosencephalon. At the beginning of cortical neurogenesis (6 gw), proliferating VZ cells labeled either with neuronal markers (SMI‐31, MAP2, β‐III‐tubulin), double‐labeled 4A4+/SMI‐31+ cells, or cells not labeled with these antibodies, were in close proximity to each other. At midgestation (17–24 gw), RG divisions were less frequent, but were spread throughout the entire cerebral cortex, including the subventricular and intermediate zones and the subpial granular layer. Several subtypes of RG were co‐labeled with vimentin and other glial markers (BLBP, GFAP, or GLAST) and quantified in vitro. In conclusion, the diversity of cortical progenitors in the human brain may, in part, explain the unique complexity of the human cerebral cortex. © 2005 Wiley‐Liss, Inc.
Calcium‐activated chloride channels in müller cells acutely isolated from tiger salamander retina
Authors: Welch Nicole C. , Lalonde Melanie R. , Barnes Steven , Kelly Melanie E.M. 
Published: September 2005

AbstractCa2+‐activated chloride channels were identified with whole‐cell patch‐clamp recording techniques in salamander retinal Müller cells. Cl(Ca) channels were activated by membrane depolarizations that elicited Ca2+ influx or the application of the Ca2+ ionophore, ionomycin. The Ca channel blocker, Cd2+, abolished the Cl(Ca) channel tail currents. Increasing the duration of the depolarizing pulse resulted in enhancement of the Cl(Ca) channel tail current. Repetitive depolarizations with rapid pulses to +20 mV produced a buildup of ICl(Ca), which reversed at 0 mV in symmetrical [Cl−] and at −40 mV when intracellular [Cl−] was reduced to 10% of the external concentration. ICl(Ca) was blocked by the Cl channel blocker niflumic acid, while niflumic acid had no effect on voltage‐gated Ca channels. These results offer the first demonstration of Cl(Ca) channels in a nonastrocytic glial cell and expand our understanding of the functional capacities of retinal glial cells. © 2005 Wiley‐Liss, Inc.