bluuebunny/crossref_metadata_2025 huggingface dataset:
A robust meta‐classification strategy for cancer detection from MS data
Authors: Bhanot Gyan , Alexe Gabriela , Venkataraghavan Babu , Levine Arnold J
Published: December 2005

We propose a novel method for phenotype identification involving a stringent noise analysis and filtering procedure followed by combining the results of several machine learning tools to produce a robust predictor. We illustrate our method on SELDI‐TOF MS prostate cancer data (http://home.ccr.cancer.gov/ncifdaproteomics/ppatterns.asp). Our method identified 11 proteomic biomarkers and gave significantly improved predictions over previous analyses with these data. We were able to distinguish cancer from non‐cancer cases with a sensitivity of 90.31% and a specificity of 98.81%. The proposed method can be generalized to multi‐phenotype prediction and other types of data (e.g., microarray data).
Morphology and gas barrier properties of polyethylene‐based nanocomposites
Authors: Jacquelot E. , Espuche E. , Gérard J.‐F. , Duchet J. , Mazabraud P.
Published: December 2005

Nanocomposites were processed by melt blending two reference matrices, a metallocene polyethylene and a low density maleic anhydride‐grafted polyethylene with an organo‐modified montmorillonite. It was shown that the introduction of a maleated polyethylene compatibilizer was required to improve the clay nanoplatelet dispersion in the metallocene polyethylene‐based nanocomposites. Increasing the montmorillonite content led to a significant increase of the barrier properties. Interfacial agents such as oxidized paraffins were shown to be more effective to reduce the gas permeability than maleated polyethylene and the dependence of the gas transport properties was discussed not only as a function of the clay dispersion but also as a function of the clay/compatibilizer and compatibilizer/matrix interactions. © 2005 Wiley Periodicals, Inc. J Polym Sci Part B: Polym Phys 44: 431–440, 2006
Fine structure and phase transition behavior of fluorinated comb copolymers
Authors: Masuya Ryosuke , Ninomiya Naoya , Fujimori Atsuhiro , Nakahara Hiroo , Masuko Toru
Published: December 2005

The fine structure in the solid state and phase transition behavior of newly synthesized comb copolymers having fluorocarbon and hydrocarbon side‐chains were investigated by temperature controlled wide angle X‐ray diffraction (WAXD) and differential scanning calorimetry (DSC). From the WAXD profiles, two kinds of short spacing peaks based on the formation of the subcell for fluorinated and hydrogenated side‐chains were confirmed at 5.0 and 4.1 Å, respectively. Furthermore, two kinds of endothermic peaks, which corresponded to melting peaks of both side‐chain crystals, appeared in heating process of the DSC thermograms. From these experimental findings, the phase separation structure having the independently packed immiscible side‐chain crystalline was formed in the whole polymer crystal. In addition, it was found that these comb polymers formed highly ordered (double) layer structure estimated using WAXD and small angle X‐ray scattering (SAXS). These fluorinated comb copolymers form a monolayer on the water surface and their transferred film with phase‐separated structure at nanometer size on solid. There were hydrogenated domains at 10–20 nm diameter scales in these phase separated surface structure of monolayers. From these experimental results, these copolymer monolayers are expected to be used as a new molecular device such as nanolithography based on the surface patterning of polymer nanomaterials. © 2005 Wiley Periodicals, Inc. J Polym Sci Part B: Polym Phys 44: 416–425, 2006
Surface properties of binary blend films of poly(N‐vinyl‐2‐pyrrolidone) and poly(vinyl alcohol) with sodium alginate
Authors: Çaykara Tuncer , Demirci Serkan , Eroğlu Mehmet S. , Güven Olgun
Published: December 2005

Blends of poly(N‐vinyl‐2‐pyrrolidone) (PVP) and poly(vinyl alcohol) (PVA) with sodium alginate (NaAlg) were prepared via casting from aqueous solutions. All the obtained blend films were optically clear to the naked eye. The surface properties of the films were analyzed by atomic force microscopy (AFM) and contact‐angle measurements. The surface free energy components of the blend films were calculated from contact‐angle data of various liquids with the Van Oss–Good methodology. It was determined that both the Lifshitz–van der Waals component and the electron‐donor component of the surface free energy of the blend films decreased with an increase in the NaAlg content to a considerable extent. However, the electron‐acceptor component of these blends increased with increasing NaAlg content. The surfaces of both the PVP/NaAlg and PVA/NaAlg blends were enriched in the low surface free energy component, that is, NaAlg. This conclusion was further confirmed by AFM image observation of the surface morphology of these blends. © 2005 Wiley Periodicals, Inc. J Polym Sci Part B: Polym Phys 44: 426–430, 2006
Strengthening of structures with advanced FRPs
Authors: Triantafillou T C
Published: December 2005

This review highlights the basic concepts involved in using a relatively new class of civil engineering materials, namely fibre‐reinforced polymers (FRP), as strengthening materials of reinforced concrete (RC), masonry and other structures. After a brief introduction to FRP, analysis, design and practical execution concepts are described first for the flexural, shear and axial capacity strengthening of RC elements using epoxy‐bonded FRP sheets. Similar concepts are also presented for masonry, which may be strengthened using either bonded or unbonded FRP. A brief reference is also made to other structures (timber, steel) and field applications. Finally, conclusions are drawn, focusing on the applicability of FRP in structural rehabilitation.
Concrete‐filled fabricated steel box columns for multistorey buildings: behaviour and design
Authors: Uy Brian
Published: December 2005

This article reviews the behaviour and design of short concrete‐filled steel box columns in multistorey buildings for gravity loading. The development of these columns is discussed and some major building projects which have utilized this concept are described. A thorough review of current research of these columns is given which highlights the construction, service and ultimate load behaviour. The paper addresses the fundamental behaviour of concrete‐filled steel columns and then summarizes the approach of current international standards for their design. Design recommendations for all the pertinent loading cases are given and further research is also suggested to supplement international code requirements.
Wind loading of structures: application of probabilistic methods
Authors: Holmes J D
Published: December 2005

This article reviews the application of probability theory and statistical analysis to wind loading of structures, a field which has developed considerably during the last 30 years. The article contains major sections on: prediction of design wind speeds, use of random process models in wind engineering, wind loads and structural reliability, and future directions. An extensive list of 60 important references and reading material has been compiled from the now vast literature.
Green's function calculations for semi‐infinite carbon nanotubes
Authors: John D. L. , Pulfrey D. L.
Published: December 2005

In the modeling of nanoscale electronic devices, the non‐equilibrium Green's function technique is gaining increasing popularity. One complication in this method is the need for computation of the “self‐energy” functions that account for the interactions between the active portion of a device and its leads. In the one‐dimensional case, these functions may be computed analytically. In higher dimensions, a numerical approach is required. In this work, we generalize earlier methods that were developed for tight‐binding Hamiltonians, and present results for the case of a carbon nanotube. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Investigation of prolate and near spherical geometries of mid‐sized silicon clusters
Authors: Marim L. R. , Lemes M. R. , Dal Pino A.
Published: December 2005

We have applied a new strategy to search for the ground‐state geometry of silicon clusters. It is a knowledge‐based procedure that severely restricts the search space. Detailed analysis of the geometry of small clusters indicates the ubiquitous presence of certain structural elements. These elements can be created from smaller units already present in a cluster. Such investigation leads to the proposal of assembly procedures (CAP's). Starting with optimized structures of small clusters, we apply one or more CAP's to create larger clusters. This approach generates the initial population for a genetic algorithm. This procedure is tested on mid‐sized silicon clusters because they show an intriguing coexistence of two different shapes: a prolate and a more spherical structures. Our method proved so efficient that for most test cases Sin(7 ≤n≤ 10 and 14 ≤n≤ 18) a simple local optimization procedure could find almost degenerate clusters of both shapes. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Transversal confined polar optical phonons in spherical quantum‐dot/quantum‐well nanostructures
Authors: Comas F. , Trallero‐Giner C. , Prado S. J. , Marques G. E. , Roca E.
Published: December 2005

Confined polar optical phonons are studied in a spherical quantum‐dot/quantum‐well (QD/QW) nanostructure by using an approach that takes into account the coupling of electromechanical oscillations and is valid in the long‐wave limit. This approach was developed a few years ago and provides results beyond the usually applied dielectric continuum approach (DCA), where just the electric aspect of the oscillations is considered. In the present paper we limit ourselves to the study of the so‐called uncoupled modes, having a purely transversal character and not involving an electric potential. We display the dispersion curves for the frequencies considering three possible nanostructures, which show different bulk phonon curvatures near the Brillouin zone Γ‐point and have been actually grown: ZnS/CdSe, CdSe/CdS and CdS/HgS. A detailed discussion of the results obtained is made, emphasizing the novelties provided by our treatment and the relevance of infrared spectroscopy in the characterization of the geometrical features of the QD/QW nanostructure. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Magnetic properties of TM3[Cr(CN)6]2·n H2O
Authors: Zentková M. , Mihalik M. , Kováč J. , Zentko A. , Mitróová Z. , Lukáčová M. , Kavečanský V. , Kiss L. F.
Published: December 2005

Magnetization measurements performed on Prussian blue analogs TM2+3[CrIII(CN)6]2·n H2O (TM = Cr, Mn, Fe, Co, Ni, Cu) confirmed the dual character of the exchange interaction (antiferromagnetic AFM and ferromagnetic FM) in this system. AFM interaction dominates for the Cr2+ sample and with rising atomic number Z the FM interaction becomes more important reaching pure FM character for the Cu2+ sample. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Sequence dependence of electronic transport in DNA
Authors: Rodríguez Antonio , Römer Rudolf A. , Turner Matthew S.
Published: December 2005

We study electronic transport in long DNA chains using the tight‐binding approach for a ladder‐like model of DNA. We find insulating behavior with localizaton lengths ξ ≈ 25 in units of average base‐pair seperation. Furthermore, we observe small, but significant differences between λ‐DNA, centromeric DNA, promoter sequences as well as random‐ATGC DNA. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Darstellung und Kristallstruktur von Diamminmagnesiumdiazid Mg(NH3)2(N3)2
Authors: Karau Friedrich , Schnick Wolfgang
Published: December 2005

Preparation and Crystal Structure of Diammin Magnesium Diazide Mg(NH3)2(N3)2

Diammin magnesium diazide was synthesized from Mg3N2 and NH4N3 in liquid ammonia and crystallized at 150 °C under autogenous atmosphere of HN3 and NH3 using sealed ampoules. Mg(NH3)2(N3)2 is a colorless, microcrystalline powder which can detonate above 180 °C. Caution, preparation and manipulation of Mg(NH3)2(N3)2 is very dangerous! The crystal structure was solved from powder data using the Patterson method and a Rietveld refinement was performed (Mg(NH3)2(N3)2, I 4/m, no. 87; a = 6.3519(1), c = 7.9176(2) Å; Z = 2, R(F2)= 0.1162). The crystal structure of Mg(NH3)2(N3)2 is related to that of SnF4. It consists of planes built up from corner sharing Mg(NH3)2(N3)4 octahedra connected equatorially over their four azide bridges with the ammonia ligands being in trans position. IR data were collected and interpreted in accordance with the structural data.
Synthese, Kristallstruktur und festkörper‐NMR‐spektroskopische Untersuchung neuer Oxonitridosilicate der Mischkristallreihe Ba4−xCaxSi6N10O
Authors: Stadler Florian , Oeckler Oliver , Schnick Wolfgang
Published: December 2005

The new oxonitridosilicates Ba4−xCaxSi6N10O have been synthesized by means of high‐temperature synthesis in a radio‐frequency furnace, starting from calcium, barium, silicon diimide and amorphous silicon dioxide. The maximum reaction temperature was at about 1450 °C. The solid solution series Ba4−xCaxSi6N10O with a phase width 1.81 ≤ x ≤ 2.95 was obtained. The crystal structure of Ba1.8Ca2.2Si6N10O was determined by X‐ray single‐crystal structure determination (P213, no. 198), a = 1040.2(1) pm, Z = 4, wR2 = 0.082). It can be described as a highly condensed network of corner‐sharing SiN4 and SiON3 tetrahedra, the voids of which are occupied by the alkaline earth ions. The structure is isotypic with that of BaEu(Ba0.5Eu0.5)YbSi6N11. In the 29Si solid‐state MAS‐NMR spectrum two isotropic resonances at −50.0 and −53.6 ppm were observed.
Syntheses and Spectroscopic Study of Some New N‐4‐Fluorobenzoyl Phosphoric Triamides; Crystal Structures of 4‐F‐C6H4C(O)N(H)P(O)R2, R = NH‐C(CH3)3, NH‐CH2C6H5, N(CH3)(CH2C6H5)
Authors: Gholivand Khodayar , Shariatinia Zahra , Pourayoubi Mehrdad
Published: December 2005

Some new N‐4‐Fluorobenzoyl phosphoric triamides with formula 4‐F‐C6H4C(O)N(H)P(O)X2, X = NH‐C(CH3)3 (1), NH‐CH2‐CH=CH2 (2), NH‐CH2C6H5 (3), N(CH3)(C6H5) (4), NH‐CH(CH3)(C6H5) (5) were synthesized and characterized by 1H, 13C, 31P NMR, IR and Mass spectroscopy and elemental analysis. The structures of compounds 1, 3 and 4 were investigated by X‐ray crystallography. The P=O and C=O bonds in these compounds are anti. Compounds 1 and 3 form one dimensional polymeric chain produced by intra‐ and intermolecular ‐P=O···H‐N‐ hydrogen bonds. Compound 4 forms only a centrosymmetric dimer in the crystalline lattice via two equal ‐P=O···H‐N‐ hydrogen bonds. 1H and 13C NMR spectra show two series of signals for the two amine groups in compound 1. This is also observed for the two α‐methylbenzylamine groups in 5 due to the presence of chiral carbon atom in molecule. 13C NMR spectrum of compound 4 shows that 2J(P,Caliphatic) coupling constant for CH2 group is greater than for CH3 in agreement with our previous study. Mass spectra of compounds 1‐3 (containing 4‐F‐C6H4C(O)N(H)P(O) moiety) indicate the fragments of amidophosphoric acid and 4‐F‐C6H4CN+ that formed in a pseudo McLafferty rearrangement pathway. Also, the fragments of aliphatic amines have high intensity in mass spectra.
Syntheses and Characterizations of Two Palladium(II) Complexes of 5‐Mercapto‐1‐methyltetrazole
Authors: Wang Yu‐Ling , Shi Qian , Bi Wen‐Hua , Li Xing , Cao Rong
Published: December 2005

Reaction of PdCl2(CH3CN)2 with the sodium salt of 5‐mercapto‐1‐methyltetrazole (MetzSNa) in methanol solution affords an interesting dinuclear palladium complex [Pd2(MetzS)4 ] (1). However, treatment of PdCl2(CH3CN)2 with neutral MetzSH ligand in methanol solution produces a mononuclear palladium complex [Pd(MetzSH)4]Cl2 (2). Both complexes were characterized by IR, 1HNMR, UV‐Vis spectroscopy as well as X‐ray crystallography. Single‐crystal X‐ray diffraction analyses of two complexes lead to the elucidation of the structures and show that 1 possesses an asymmetric structure: one Pd atom is tetracoordinated by three sulfur atoms and one nitrogen atom to form PdS3N coordination sphere, the other Pd atom is tetracoordinated by three nitrogen atoms and one sulfur atom to form PdSN3 coordination sphere. The molecules of 1 are associated to 1‐D infinite linear chain by weak intermolecular Pd···S contacts in the crystal lattice. In 2, the Pd atom lies on an inversion center and has a square‐planar coordination involving the S atoms from four MetzSH ligands. The two chloride ions are not involved in coordination, but are engaged in hydrogen bonding.
Genetic heterogeneity of membrane‐bound β‐glucosidase in Gaucher's disease
Authors: Carroll M.
Published: December 2005

Membrane‐bound β‐glucosidase of leukocytes exists in two forms, one with optimal activity at about pH 4.5, whereas the other is most active at pH 5.5–6.0. In one case of type 1 (adult) Gaucher's disease, the pH 4.5 activity was totally deficient, but the pH 5.5 activity was present in normal amounts; obligate heterozygotes had half the normal activity at pH 4.5. In two different cases, the membrane‐bound β‐glucosidase was completely absent when assayed at either pH 4.5 or pH 5.5. Two further cases had residual activity at both pH values; however, the residual enzymes had different thermostability properties than the corresponding enzymes of control leukocytes. The results are consistent with the existence of at least three genetic variants of type 1 (adult) Gaucher's disease.
Complementation analysis in fibroblasts from eight patients with clinically different forms of citrullinaemia
Authors: Kennaway N. G. , Curtis H. C.
Published: December 2005

We have examined the possibility of genetic heterogeneity in citrullinaemia by measuring the incorporation of [14C]citrulline into protein in PEG‐induced heterokaryons derived from paired combinations of fibroblasts from eight patients with varying forms of the disease. The results are compatible with interallelic, but not intergenic, complementation in several of these fusions.
Pyrimidine and purine metabolites in ornithine carbamoyl transferase deficiency
Authors: Webster D. R. , Simmonds H. A. , Barry D. M. J. , Becroft D. M. O.
Published: December 2005

Detailed biochemical studies have been carried out in a female heterozygote for ornithine carbamoyltransferase (OCT) deficiency. Increased levels of the pyrimidines, orotic acid, uridine and uracil, were observed in plasma as well as urine by utilizing an adaptation of high performance liquid chromatography (HPLC). Urinary clearances of these compounds were high, that of orotic acid indicating net secretion. Urinary uric acid clearance was also elevated, a finding attributed to the uricosuric effect of the orotic acid excreted concomitantly. The results in this child and her family are typical of OCT deficiency. They confirm considerable genetic heterogeneity in the biochemical as well as clinical expression in this defect.
Pterin metabolism in normal subjects and hyperphenylalaninaemic patients
Authors: Dhondt J. L. , Farriaux J. P. , Largilliere C. , Dautrevaux M. , Ardouin P.
Published: December 2005

The application of high performance liquid chromatography to the estimation of urinary pterins is illustrated by results from normal subjects and from patients with phenylketonuria, dihydropteridine reductase deficiency and biopterin synthetase deficiency. In normal subjects following a phenylalanine load there is a temporary increase in pterin elimination, the pattern being different to that seen in chronic hyperphenylalaninaemia.
Use of α‐methylphenylalanine for studies of brain development in experimental phenylketonuria
Authors: Huether G. , Neuhoff V.
Published: December 2005

Experimental phenylketonuria was induced in newborn rats by administration ofl‐phenylalanine and α‐methylphenylalanine. There was no primary disturbance of myelination, but reduced cell proliferation, early cell death and compensatory hyperplasia were evident in the cerebellum.
Renal transport of aromatic acids in patients with phenylketonuria
Authors: Langenbeck U. , Behbehani A. , Luthe H.
Published: December 2005

Renal clearance of phenylpyruvic acid is maximal at a plasma concentration of 40–60 µmol/l. This concentration is obtained with plasma phenylalanine concentrations of 1.0–1.2mmol/l, the threshold for separating classical phenylketonuria from phenylketonuria variants.
Histoenzymological activities of gaucher cells
Authors: Lageron A.
Published: December 2005

Seven samples from liver and/or spleen of patients with Gaucher's disease have been investigated by histoenzymological techniques. Unexpected results such as β‐glucosidase positivity, increase of other hydrolases, and increase of dehydrogenase activities are discussed.
Hypermethioninaemia and 3‐Hydroxyisobutyric aciduria in an apparently healthy baby
Authors: Congdon P. J. , Haigh D. , Smith R. , Green Anne , Pollitt R. J.
Published: December 2005

An apparently healthy baby with persistent hypermethioninaemia excretes increased amounts of 3‐hydroxyisobutyrate, 3‐hydroxypropionate, β‐aminoisobutyrate and β‐alanine. A defect in the oxidation of methylmalonic and malonic semialdehydes is proposed but the cause of the hypermethioninaemia is obscure.
The activity of carbamoyl‐phosphate synthetase I and ornithine carbamoyltransferase (OCT) in the intestine and screening for OCT deficiency in the rectal Mucosa
Authors: Matsushima A. , Orii T.
Published: December 2005

Carbamoyl‐phosphate synthetase I and ornithine carbamoyltransferase are present in small intestine and rectum. Rectal mucosa may be used in the detection of ornithine carbamoyltransferase deficiency.
Preliminary report on mellituria in phenylketonuric children: Modification in the excretion of glucose and pentoses
Authors: Piccardo M. G. , Rosa M. , Russo L.
Published: December 2005

Urine from children with phenylketonuria contains less glucose and more pentoses than urine from normal children.
Estimation of energy metabolism in human skeletal muscle homogenate as a diagnostic aid
Authors: Ruitenbeek W. , Sengers R. C. A. , Trijbels J. M. F. , Stadhouders A. M. , Janssen A. J. M.
Published: December 2005

A method is presented for thein vitro testing of the energy metabolism of human skeletal muscle. The levels of creatine phosphate, ATP, ADP and AMP, which are defined by the activity of many enzyme systems, are measured after incubation of the muscle homogenate with various substrates in the presence or absence of specific inhibitors. Detection of abnormal metabolite concentrations is important for diagnosis of patients with myopathic syndromes.
Glucose‐6‐phosphate dehydrogenase deficiency and mental retardation
Authors: Seth P. K. , Tara Devi S. , Seth S.
Published: December 2005

An increased incidence of glucose‐6‐phosphate dehydrogenase deficiency has been found in a group of mentally retarded individuals compared with a matched population of normal intelligence.
Towards enzyme therapy using carrier erythrocytes
Authors: Sprandel U. , Hubbard A. R. , Chalmers R. A.
Published: December 2005

Recent work leading to the production of resealed erythrocytes with enhancedin vivo survival is reviewed. The term ‘carrier erythrocytes’ is proposed for these cells and possible therapeutic uses are discussed.
In Vivo residual activities of the phenylalanine hydroxylating system in phenylketonuria and variants
Authors: Trefz F. K. , Bartholomé K. , Bickel H. , Lutz P. , Schmidt H. , Seyberth H. W.
Published: December 2005

The results ofin vivo determinations of phenylalanine‐hydroxylating capacity using deuterated phenylalanine loads are compared with phenylalanine hydroxylase activity in liver biopsy samples and the results of oral protein challenge.
Substrate inhibition of HGPRT: Protein structural and kinetic studies of a human variant
Authors: Steyn L. M. , Harley E. H.
Published: December 2005

Kinetic and electrophoretic properties of a new mutant form of hypoxanthine‐guanine phosphoribosyltransferase are described.
Four sanfilippo C patients in a family pedigree
Authors: Orii T. , Taga T. , Sukegawa K. , Tamanawa E.
Published: December 2005

The deficiency of acetyl‐CoA:α‐glucosaminideN‐acetyltransferase was detected in skin fibroblast homogenates from four patients in a family pedigree. Further, it was observed that the relative proportion of the acetyl groups in heparan sulphate from a Sanfilippo C patient was much lower than those obtained from Sanfilippo A and B patients.
Properties of human alkaline phosphatase and prenatal diagnosis of hypophosphatasia
Authors: Shin‐Buehring Y. S. , Santer R. , Osang M. , Schaub J.
Published: December 2005

The alkaline phosphatase activity in serum, leukocytes, amniotic fluid and cultured fibroblasts and amniotic fluid cells has been characterized by inhibition studies and by mini‐electrophoresis.
Enzymatic diagnosis of congenital lethal hypophosphatasia in tissues, plasma and diploid skin fibroblasts
Authors: Vanneuville F. J. , Leroy J. G.
Published: December 2005

Enzymatic studies onpost‐mortem tissues and on cultured fibroblasts from three patients with hypophosphatasia are presented.
Familial AMP deaminase deficiency with skeletal muscle type I atrophy and fatal cardiomyopathy
Authors: Scholte H. R. , Busch H. F. M. , Luyt‐Houwen I. E. M.
Published: December 2005

Muscular AMP deaminase deficiency was found in two sibs suffering from a skeletal myopathy, characterized by type I fibre atrophy and a dilated cardiomyopathy. The family history suggests an autosomal dominant inheritance of this disorder.
Late onset form of mucopolysaccharidosis type I: Clinical aspect and biochemical characterization of residual α‐l‐iduronidase activity
Authors: Ullrich K. , Gröbe H. , von Figura K. , Hopwood J. J. , Muller V.
Published: December 2005

Clinical and roentgenographic features of two patients with the late onset form of mucopolysaccharidosis type I are presented. The residual α‐L‐iduronidase in cultured fibroblasts had an abnormalKm which differed from that found in the early onset (Hurler) form.
3‐Hydroxy‐3‐methylglutaric aciduria combined with 3‐methylglutaconic aciduria: A new case
Authors: Divry P. , Rolland M. O. , Teyssier J. , Cotte J. , Formosinho Fernandes M. C. , Tavares de Almeida I. , da Silveira C.
Published: December 2005

The urinary organic acids of a new case of 3‐hydroxy‐3‐methylglutaryl‐CoA lyase deficiency have been analysed by gas chromatography‐mass spectrometry. This patient's fibroblasts showed a reduced ability to oxidize leucine.
Studies on cultured fibroblasts from patients with defects of biotin‐dependent carboxylation
Authors: Bartlett K. , Ng H. , Dale G. , Green A. , Leonard J. V.
Published: December 2005

Seven patients with inborn defects of biotin‐dependent enzymes are described. The results of studies of fibroblast carboxylases and an account of the problems associated with their measurement are given.
The adult presenting idiopathic fanconi syndrome
Authors: Brenton D. P. , Isenberg D. A. , Cusworth D. C. , Garrod P. , Krywawych S. , Stamp T. C. B.
Published: December 2005

The adult presenting Fanconi syndrome is a rare familial disorder. A 30‐year follow‐up of one of the original families in the literature is reported here. Two important points have emerged. Firstly, the inheritance in this family is dominant, not recessive as originally suggested, and there remains no good example in the literature of a recessive inheritance of this disorder. Second, in this family lactic aciduria and tubular proteinuria are probably the earliest manifestations of the disorder in childhood, with glycosuria and aminoaciduria developing in the second decade and osteomalacia from the start of the fourth decade. Glomerular function deteriorates slowly but is compatible with a normal lifespan.
On the Ginzburg–Landau model of a superconducting ball in a uniform field
Authors: Alama Stan , Bronsard Lia , Montero J. Alberto
Published: December 2005

We consider the three-dimensional Ginzburg–Landau model for a solid spherical superconductor in a uniform magnetic field, in the limit as the Ginzburg–Landau parameter

\kappa = 1/ ɛ\rightarrow \infty

. By studying a limiting functional, we identify a candidate for the lower critical field

H_{c_{1}}

, the value of the applied field strength at which minimizers first exhibit vortices. For applied fields of this strength we show the existence of locally minimizing solutions with vortices located along a diameter of the sphere parallel to the applied field direction. To analyze these problems we use a combination of techniques, involving least perimeter problems, weak Jacobians and rectifiable currents, and special Hodge decompositions.

Nous étudions la limite quand le paramètre de Ginzburg–Landau

\kappa = {}^{1}/_{ɛ}\rightarrow \infty

pour le modèle de Ginzburg–Landau en trois dimension dans le cas d'une boule placée dans un champ magnétique uniforme. Nous identifions une fonctionnelle limite qui nous permet de trouver le premier champ critique

H_{c_{1}}

, c'est à dire le champ au dessus duquel les minimiseurs commencent à presenter des vortex. Nous montrons qu'il existe des solutions localement minimisantes ayant des vortex le long du diamètre de la boule qui est parallèle au champ appliqué quand sa norme est de l'ordre de

H_{c_{1}}

. Nous nous servons de techniques provenant de la théorie de la mesure géométrique, incluant les jacobiens faibles et les courants rectifiables, ainsi que de techniques provenant de problèmes de minimisation de périmètre.
Global subanalytic solutions of Hamilton–Jacobi type equations
Authors: Trélat Emmanuel
Published: December 2005

In the 80's, Crandall and Lions introduced the concept of viscosity solution, in order to get existence and/or uniqueness results for Hamilton–Jacobi equations. In this work, we first investigate the Dirichlet and Cauchy–Dirichlet problems for such equations, where the Hamiltonian is associated to a problem of calculus of variations, and prove that, if the data are analytic, then the viscosity solution is moreover subanalytic. We then extend this result to Hamilton–Jacobi equations stemming from optimal control problems, in particular from sub-Riemannian geometry, which are generalized eikonal equations.

As a consequence, the set of singularities of the viscosity solutions of such Hamilton–Jacobi equations is a subanalytic stratified manifold of codimension greater than or equal to one.
Rickety Bridges: Using the Media in Deliberative Democracy
Authors: PARKINSON JOHN
Published: December 2005

It is a fairly common criticism of deliberative democratic ideals that one cannot involve thousands, let alone millions, of people in a decision-making process and still retain its deliberative character, at least not in any sense as strict as those of the classic expositions of deliberative democratic principles. Various solutions to this scale problem have been proposed, but implicit in most deliberative designs is the media solution. People can watch the debate unfold on television, can listen to it on the radio, can follow it, or even contribute to a limited extent, online. This is supposed to ensure not only that deliberation is subject to the check of publicity, but also that it is dispersed throughout the public sphere.

This is precisely the idea behind James Fishkin's deliberative poll technique, designed to be televised so that deliberation among a few would be brought to ‘an audience of millions’, ‘bridging the gap’ between informed and uninformed opinion, and acting as a ‘catalyst’ to change the nature of discussion on a topic in a broader community. But what happens when we rely on the media to bear the weight of responsibility for generating links between insiders and outsiders in public deliberation? The media are not broad pipes which simply convey whatever is put into them but, like all institutions, shape that input in sometimes significant ways. If we are to understand the value of the media in addressing problems of deliberative scale, we need to understand those institutional effects.

In this Research Note I examine the effects the media had on the kinds of information transferred from deliberators to audience in a deliberative poll that took place in the United Kingdom in July 1998.
THE ‘NATIVE’ UNDEFINED: COLONIAL CATEGORIES, ANGLO-AFRICAN STATUS AND THE POLITICS OF KINSHIP IN BRITISH CENTRAL AFRICA, 1929–38
Authors: LEE CHRISTOPHER JOON-HAI
Published: December 2005

This article examines the categorical problem that persons of ‘mixed-race’ background presented to British administrations in eastern, central and southern Africa during the late 1920s and 1930s. Tracing a discussion regarding the terms ‘native’ and ‘non-native’ from an obscure court case in Nyasaland (contemporary Malawi) in 1929, to the Colonial Office in London, to colonial governments in eastern, central and southern Africa, this article demonstrates a lack of consensus on how the term ‘native’ was to be defined, despite its ubiquitous use. This complication arrived at a particularly crucial period when indirect rule was being implemented throughout the continent. Debate centered largely around the issue of racial descent versus culture as the determining factor. The ultimate failure of British officials to arrive at a clear definition of the term ‘native’, one of the most fundamental terms in the colonial lexicon, is consequently suggestive of both the potential weaknesses of colonial state formation and the abstraction of colonial policy vis-à-vis local empirical conditions. Furthermore, this case study compels a rethinking of contemporary categories of analysis and their historical origins.
Klinghoffer in Brooklyn Heights
Authors: FINK ROBERT
Published: December 2005

Is The Death of Klinghoffer anti-Semitic? Performances of the opera at the Brooklyn Academy of Music in September 1991 were at the epicentre of a controversy that continues to this day; the New York audience was – and remains – uniquely hostile to the work. A careful reception analysis shows that New York audiences reacted vehemently not so much to an ideological position on the Israeli–Palestinian conflict, but to specific nuances in the satirical portrayal of American Jewish characters in one controversial scene later cut from the opera, a scene that must be read closely and in relation to specifically American-Jewish questions of ethnic humour, assimilation, identity and multiculturalism in the mass media. I understand the opera's negative reception in the larger context of the increasingly severe crises that beset American Jewish self-identity during the Reagan-Bush era. Ultimately the historical ability of Jews to assimilate through comedy, to ‘enter the American culture on the stage laughing’, in Leslie Fiedler's famous formulation, will have to be reconsidered. A close reading of contested moments from the opera shows librettist Alice Goodman and composer John Adams avoiding the romance of historical self-consciousness as they attempt to construct a powerful yet subtle defence of the ordinary and unassuming.
Sustainable community and environment in tropical Singapore high-rise housing: the case of Bedok Court condominium
Authors: Bay Joo-Hwa
Published: December 2005

Is it possible to create successful ‘sky gardens’ – spaces which encourage outdoor living and communal interaction – as part of high-rise high-density apartment developments?
Molecular pathogenesis of rheumatic fever and rheumatic heart disease
Authors: Guilherme Luiza , Faé Kellen , Oshiro Sandra E. , Kalil Jorge
Published: December 2005

Molecular mimicry between streptococcal and human proteins has been proposed as the triggering factor leading to autoimmunity in rheumatic fever (RF) and rheumatic heart disease (RHD). This article summarises studies on genetic susceptibility markers involved in the development of RF/RHD. It also focuses on the molecular mimicry in RHD mediated by the responses of B and T cells of peripheral blood, and T cells infiltrating heart lesions, against streptococcal antigens and human tissue proteins. The molecular basis of T-cell recognition is assessed through the definition of heart-crossreactive antigens. The production of cytokines from peripheral and heart-infiltrating mononuclear cells suggests that T helper 1 (Th1)-type cytokines are the mediators of RHD heart lesions. An insufficiency of interleukin 4 (IL-4)-producing cells in the valvular tissue might contribute to the maintenance and progression of valve lesions.
Comparison of the reflectance and Crystal Violet assays for measurement of biofilm formation by Enterococcus
Authors: Broschat S. L. , Call D. R. , Kuhn E. A. , Loge F. J.
Published: December 2005

In previous work, we described a protocol for measuring biofilm formation on opaque and non-opaque surfaces using reflected light. In the present work, we used the reflectance assay to assess biofilm formation for 14 strains of Enterococcus and compared our results with those obtained using a Crystal Violet (CV) assay. In general, the results for the two assays were in agreement: both identified the same Enterococcus strain as the highest biofilm-forming strain and the same two strains as the lowest biofilm-forming strains. However, two Enterococcus strains identified as high biofilm formers by the CV assay were not identified as such by the reflectance assay. A single numerical value was obtained for each of the reflectance assay results that corresponded well with the confluence of a biofilm (and presumably its depth) using phase contrast microscopy.
Parametric study of shock‐induced transport and energization of relativistic electrons in the magnetosphere
Authors: Gannon J. L. , Li X. , Temerin M.
Published: December 2005

The sudden appearance of a new radiation belt consisting of electrons with energies greater than 13 MeV near 2.5 Earth radii (RE), was observed by CRRES (Combined Radiation and Release Experiment Satellite) on 24 March 1991. Li et al. (1993) showed that the cause was an electromagnetic pulse within the Earth's magnetosphere caused by an unusually strong, fast shock in the solar wind. Sudden shock‐induced injections of electrons with energies above 10 MeV to equatorial distances within 2.5 RE are extremely rare because of the intensity of the shock required. In the current study, the propagation velocity parameter and electric field amplitude of pulses within the magnetosphere in the Li et al. model were varied from 750 to 2500 km/s and 70 to 400 mV/m, respectively. It was found that a stronger electric field shifted the peak of the resultant relativistic electron population toward the Earth. Doubling the electric field amplitude from 120 to 240 mV/m moved the peak of the injected electrons with energies above 13 MeV from 2.8 to 2.4 RE. However, as the electric field pulse becomes even larger, the increase in response diminishes. This asympotic behavior shows that it is extremely difficult to produce energetic electron injections inside two Earth radii. The nominal propagation velocity (velocity parameter) is compared to the radial propagation velocities that would have been measured under this model and others and compared to observation. It is found that although the model radial velocity is smaller than the velocity parameter and decreasing with radial distance, it is faster than MHD results and observations. Decreasing the nominal propagation velocity of the pulse within the magnetosphere from 2500 km/s to 1400 km/s also moved the peak of the injected electrons with energies above 13 MeV slightly closer to the Earth. However, at velocities smaller than approximately 1200 km/s the number of electrons injected within 2.5 Earth radii with energies above 13 MeV greatly decreased. Halving the velocity from 2000 to 1000 km/s shifted the peak of electrons with energy greater than 13 MeV from L = 2.6 to L = 2.4 but produced a count rate reduced by a factor of more than 250, resulting in no significant new radiation belt. These results show that the typical large electromagnetic impulses caused by interplanetary shocks, with amplitudes of the order of 10 mV/m, are more than an order of magnitude too small to produce any significant new radiation belts within 2.5 Earth radii with energies of the order of 10 MeV. Contributing to the difficulty in producing such a new belt is the need for an already relativistic electron population with adequate flux beyond geosynchronous orbit. These results thus also help explain the rarity of events such as the 24 March 1991 injection.
Efficient methods for finding transition states in chemical reactions: Comparison of improved dimer method and partitioned rational function optimization method
Authors: Heyden Andreas , Bell Alexis T. , Keil Frerich J.
Published: December 2005

A combination of interpolation methods and local saddle-point search algorithms is probably the most efficient way of finding transition states in chemical reactions. Interpolation methods such as the growing-string method and the nudged-elastic band are able to find an approximation to the minimum-energy pathway and thereby provide a good initial guess for a transition state and imaginary mode connecting both reactant and product states. Since interpolation methods employ usually just a small number of configurations and converge slowly close to the minimum-energy pathway, local methods such as partitioned rational function optimization methods using either exact or approximate Hessians or minimum-mode-following methods such as the dimer or the Lanczos method have to be used to converge to the transition state. A modification to the original dimer method proposed by [Henkelman and Jónnson J. Chem. Phys. 111, 7010 (1999)] is presented, reducing the number of gradient calculations per cycle from six to four gradients or three gradients and one energy, and significantly improves the overall performance of the algorithm on quantum-chemical potential-energy surfaces, where forces are subject to numerical noise. A comparison is made between the dimer methods and the well-established partitioned rational function optimization methods for finding transition states after the use of interpolation methods. Results for 24 different small- to medium-sized chemical reactions covering a wide range of structural types demonstrate that the improved dimer method is an efficient alternative saddle-point search algorithm on medium-sized to large systems and is often even able to find transition states when partitioned rational function optimization methods fail to converge.
Discrete particle noise in particle-in-cell simulations of plasma microturbulence
Authors: Nevins W. M. , Hammett G. W. , Dimits A. M. , Dorland W. , Shumaker D. E.
Published: December 2005

Recent gyrokinetic simulations of electron temperature gradient (ETG) turbulence with the global particle-in-cell (PIC) code GTC [Z. Lin et al., Proceedings of the 20th Fusion Energy Conference, Vilamoura, Portugal, 2004 (IAEA, Vienna, 2005)] yielded different results from earlier flux-tube continuum code simulations [F. Jenko and W. Dorland, Phys. Rev. Lett. 89, 225001 (2002)] despite similar plasma parameters. Differences between the simulation results were attributed to insufficient phase-space resolution and novel physics associated with global simulation models. The results of the global PIC code are reproduced here using the flux-tube PIC code PG3EQ [A. M. Dimits et al., Phys. Rev. Lett. 77, 71 (1996)], thereby eliminating global effects as the cause of the discrepancy. The late-time decay of the ETG turbulence and the steady-state heat transport observed in these PIC simulations are shown to result from discrete particle noise. Discrete particle noise is a numerical artifact, so both these PG3EQ simulations and, by inference, the GTC simulations that they reproduced have little to say about steady-state ETG turbulence and the associated anomalous heat transport. In the course of this work several diagnostics are developed to retrospectively test whether a particular PIC simulation is dominated by discrete particle noise.
Thermal breaking of spanning water networks in the hydration shell of proteins
Authors: Brovchenko I. , Krukau A. , Smolin N. , Oleinikova A. , Geiger A. , Winter R.
Published: December 2005

The presence of a spanning hydrogen-bonded network of water at the surface of biomolecules is important for their conformational stability, dynamics, and function. We have studied by computer simulations the clustering and percolation of water in the hydration shell of a small elastinlike peptide (ELP) and the medium-size protein staphylococcal nuclease (SNase), in aqueous solution. We have found that in both systems a spanning network of hydration water exists at low temperatures and breaks up with increasing temperature via a quasi-two-dimensional percolation transition. The thermal breaking of the spanning water network occurs at biologically relevant temperatures, in the temperature range, which is close to the temperature of the “inverse temperature transition” of ELP and the unfolding temperature of SNase, respectively.
Molecular elimination in photolysis of o- and p-fluorotoluene at 193 nm: Internal energy of HF determined with time-resolved Fourier transform spectroscopy
Authors: Yang Sheng-Kai , Liu Suet-Yi , Chen Hui-Fen , Lee Yuan-Pern
Published: December 2005

Following the photodissociation of o-fluorotoluene [o-C6H4(CH3)F] at 193 nm, rotationally resolved emission spectra of HF(1⩽v⩽4) in the spectral region of 2800–4000cm−1 are detected with a step-scan Fourier transform spectrometer. HF(v⩽4) shows nearly Boltzmann-type rotational distributions corresponding to a temperature ∼1080K; a short extrapolation from data in the period of 0.5–4.5μs leads to a nascent rotational temperature of 1130±100K with an average rotational energy of 9±2kJmol−1. The observed vibrational distribution of (v=1):(v=2):(v=3)=67.6: 23.2: 9.2 corresponds to a vibrational temperature of 5330±270K. An average vibrational energy of 25±312kJmol−1 is derived based on the observed population of HF(1⩽v⩽3) and estimates of the population of HF (v=0 and 4) by extrapolation. Experiments performed on p-fluorotoluene [p-C6H4(CH3)F] yielded similar results with an average rotational energy of 9±2kJmol−1 and vibrational energy of 26±312kJmol−1 for HF. The observed distributions of internal energy of HF in both cases are consistent with that expected for four-center elimination. A modified impulse model taking into account geometries and displacement vectors of transition states during bond breaking predicts satisfactorily the rotational excitation of HF. An observed vibrational energy of HF produced from fluorotoluene slightly smaller than that from fluorobenzene might indicate the involvement of seven-membered-ring isomers upon photolysis.
Scaling of mesoscale simulations of polymer melts with the bare friction coefficient
Authors: Kindt P. , Briels W. J.
Published: December 2005

Both the Rouse and reptation model predict that the dynamics of a polymer melt scale inversely proportional with the Langevin friction coefficient ξ. Mesoscale Brownian dynamics simulations of polyethylene validate these scaling predictions, providing the reptational friction ξR=ξ+ξC is used, where ξC reflects the fundamental difference between a deterministic and a stochastic propagator even in the limit of ξ to zero. The simulations have been performed with Langevin background friction and with pairwise friction, as in dissipative particle dynamics. Both simulation methods lead to equal scaling behavior with ξC having almost the same value in both cases. The scaling is tested for the diffusion g(t), the shear relaxation modulus G(t), and the Rouse mode autocorrelations of melts of C120H242, C400H802, and C1000H2002. The derived dynamical scaling procedure is very useful to reduce run-time in mesoscale computer simulations, especially if pairwise friction is applied.