bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mouse imprinting defect mutations that model Angelman syndrome
Authors: Wu Mei‐Yi , Chen Ken‐Shiung , Bressler Jan , Hou Aihua , Tsai Ting‐Fen , Beaudet Arthur L. 
Published: January 2006

AbstractPrader‐Willi syndrome (PWS) and Angelman syndrome (AS) are neurobehavioral disorders resulting from deficiency of imprinted gene expression from paternal or maternal chromosome 15q11‐15q13, respectively. In humans, expression of the imprinted genes is under control of a bipartite cis‐acting imprinting center (IC). Families with deletions causing PWS imprinting defects localize the PWS‐IC to 4.3 kb overlapping with SNRPN exon 1. Families with deletions causing AS imprinting defects localize the AS‐IC to 880 bp 35 kb upstream of the PWS‐IC. We report two mouse mutations resulting in defects similar to that seen in AS patients with deletion of the AS‐IC. An insertion/duplication mutation 13 kb upstream of Snrpn exon 1 resulted in lack of methylation at the maternal Snrpn promoter, activation of maternally repressed genes, and decreased expression of paternally repressed genes. The acquisition of a paternal epigenotype on the maternal chromosome in the mutant mice was demonstrated by the ability to rescue the lethality and growth retardation in a mouse model of a PWS imprinting defect. A second mutation, an 80‐kb deletion extending upstream of the first mutation, caused a similar imprinting defect with variable penetrance. These results suggest that there is a mouse functional equivalent to the human AS‐IC. genesis 44:12–22, 2006. © 2006 Wiley‐Liss, Inc.
Effect of filler‐to‐matrix blending ratio on the mechanical strength of palm‐based biocomposite boards
Authors: Badri Khairiah Haji , Mat Amin Khairul Anuar , Othman Zulkefly , Manaf Hairani Abdul , Khalid Nur Khairani 
Published: January 2006

AbstractThe effect of the blending ratio of a polyurethane matrix and oil‐palm empty fruit bunch (EFB) fibers on the mechanical properties of biocomposite boards has been studied. The PU matrix and EFB fibers were used at blending ratios of 25:75, 30:70 and 35:65 (by weight). The mechanical property of hardness was studied. The intention of this study was to produce fiberboard from a vegetable oil‐based polyester as the matrix and biomass from the palm oil industry, namely EFB. It was found that the blending ratio with a lower filler loading (35:65) gave higher impact and flexural strengths due to better fiber encapsulation which enhanced the fiber–matrix interfacial adhesion. Copyright © 2005 Society of Chemical Industry
Morphology, thermal and mechanical behavior of polypropylene nanocomposites toughened with poly(ethylene‐co‐octene)
Authors: Lim Jian Wei , Hassan Azman , Rahmat Abdul Razak , Wahit Mat Uzir 
Published: January 2006

AbstractRubber‐toughened polypropylene (PP) nanocomposites containing organophilic layered silicates were prepared by means of melt extrusion at 230 °C using a co‐rotating twin‐screw extruder in order to examine the influence of the organoclay and the addition of PP grafted with maleic anhydride (PPgMAH) as a compatibilizer on the morphological, mechanical and thermal properties. The mechanical properties of rubber‐toughened polypropylene nanocomposites (RTPPNCs) were studied through tensile, flexural and impact tests. Scanning electron microscopy (SEM) was used for investigation of the phase morphology and rubber particles size. X‐ray diffraction (XRD) was employed to characterize the formation of nanocomposites. The thermal properties were investigated by using thermogravimetric analysis (TGA) and differential scanning calorimetry (DSC). The dynamic mechanical properties were examined by using dynamic mechanical analysis (DMA). From the tensile and flexural tests, the optimum loading of organoclay in RTPP was found to be 6 wt%. The optimum loading of PPgMAH, based on the tensile and flexural properties, was also 6 wt%. The increase in the organoclay and PPgMAH content resulted in a severe embrittlement, manifested by a drop in the impact strength and tensile elongation at break. XRD studies revealed that intercalated RTPPNCs had been successfully prepared where the macromolecular PP segments were intercalated into the interlayer space of the organoclay. In addition, the organoclay was dispersed more evenly in the RTPPNC as the PPgMAH content increased. TGA results revealed that the thermal stability of the RTPPNC improved significantly with the addition of a small amount of organoclay. Copyright © 2006 Society of Chemical Industry
Correlation of the rates of solvolysis of 2‐furancarbonyl chloride and three naphthoyl chlorides
Authors: D'Souza Malcolm J. , Boggs Mary E. , Kevill Dennis N. 
Published: January 2006

AbstractThe correlations of the specific rates of solvolysis of the title compounds using extended forms of the Grunwald–Winstein equation are consistent with the overall picture which is emerging for acyl chloride solvolyses, with competing addition–elimination (with rate‐determining addition) and ionization (assisted by nucleophilic solvation) pathways. Except in the more ionizing solvents of low nucleophilicity, 2‐furancarbonyl chloride follows the addition–elimination pathway, in contrast to 2‐thiophenecarbonyl chloride. Except in solvents of highest nucleophilicity and low ionizing power, the solvolyses of the naphthoyl chlorides (1‐naphthoyl, 2‐naphthoyl and 6‐methyl‐2‐naphthoyl) all favor the ionization pathway. In the correlation of the 1‐naphthoyl chloride solvolyses, there is a slight improvement when a term governed by the sensitivity to changes in the aromatic ring parameter (hI) is incorporated; this can be associated with a rather minor steric hindrance involving the peri‐hydrogen. Copyright © 2006 John Wiley & Sons, Ltd.
Selectively sulfonated poly(aryl ether sulfone)‐b‐polybutadiene for proton exchange membrane
Authors: Zhang Xingpeng , Liu Shengzhou , Yin Jie 
Published: January 2006

AbstractA series of selectively sulfonated poly(arylene ether sulfone)‐b‐polybutadiene copolymers (SPAES‐b‐PB) were prepared based on carboxyl terminated polybutadiene (CTPB) and sulfonated poly(arylene ether sulfone) (SPAES) that was directly prepared by polycondensation of 4,4′‐isopropylidenediphenol with different molar ratios of disodium 3,3′‐disulfonate‐4,4′‐dichlorodiphenyl sulfone (SDCDPS) to 4,4′‐dichlorodiphenylsulfone (DCDPS), and subsequent selective postsulfonation of flexible PB block was carried out. Epoxidized modification of membranes was conducted by an in situ‐generated peracid method. The content of sulfonic acid groups attaching to aromatic rings in SPAES was determined by 1H NMR and was in good aggrement with the controlled ratios. The effect of sulfonated rigid blocks on the postsulfonation of PB blocks was studied by Fourier transform infrared spectroscopy. The glass transition temperature (Tg) and the temperature of the melting peak (T) of membranes in acid form were studied by differential scanning calorimetry. Fenton's reagent test revealed that the selectively sulfonated SPAES‐b‐PB membranes had good stability to oxidation. The microstructure of rod‐like rigid SPAES blocks and interpenetrating network of ions were observed by transmission electron microscopy. Complex impedance measurement showed that an epoxidized membrane with SPAES‐40 exhibited the highest proton conductivity (1.08 × 10−1 S/cm, 90 °C), which was due to the formation of obvious ionic networks. © 2006 Wiley Periodicals, Inc. J Polym Sci Part B: Polym Phys 44: 665–672, 2006
Exact exchange for correlated electrons
Authors: Novák P. , Kuneš J. , Chaput L. , Pickett W. E. 
Published: January 2006

AbstractA density functional based “exact exchange for correlated electrons” (EECE) method of electronic structure determination, intended as an improved initial point for the description of systems containing correlated electrons, is proposed and its implementation is described. This approach bears strong resemblance to certain methods already in use: in implementation it is much like the LDA+U method with the important distinction that it is parameter‐free; in concept has much in common with previous ‘exact exchange’ and self‐interaction corrected approaches. The EECE method is applied to several systems with varying correlation strengths and characters (FeF2, NiO, hcp Gd, FeAl) and the results are compared with those obtained by LDA +U and Hartree–Fock methods as well as results within the local spin density and generalized gradient approximations to density functional theory. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Ab initio and DFT study of Y3+ hydration
Authors: Buzko Vladimir Y. , Sukhno Igor V. , Buzko Margarita B. , Subbotina Julia O. 
Published: January 2006

AbstractAn ab initio study of the molecular geometry and stability of Y(H2O) (n = 1–10) clusters at the restricted Hartree–Fock (RHF) and second‐order Møller–Plesset (MP2) levels is reported. The results obtained indicate that in aqueous solutions, the Y3+ ion is primarily 8‐coordinate. The most popular DFT functionals were tested to calculate the dimensions of the Y(H2O) aqua ion. It was found that SVWN5, SPL, BHandH, B3P86, and PBE1PBE functionals give the closest results to the experimental YO distance. © 2006 Wiley Periodicals, Inc. Int J Quantum Chem, 2006
Analysis of oxidizer salt mixtures by electrospray ionization mass spectrometry
Authors: Sigman Michael E. , Armstrong Paul 
Published: January 2006

AbstractBinary aqueous mixtures of NaNO3, KNO3 and NaClO4 oxidizers were analyzed using electrospray ionization mass spectrometry. Sodium nitrate solutions were observed to form doubly charged clusters of the type [(NaNO3)n2Na]2+ and [(NaNO3)n2NO3]2−, where n = 11, 13, 15, etc., in addition to singly charged cluster ions that have been reported previously. The identity of the doubly charged clusters was determined by tandem mass spectrometry. Two‐component NaNO3KNO3 salt solutions were observed to form cluster ions of the type [(NaNO3)i(KNO3)jNO3]− in the negative ion mode and [(NaNO3)i(KNO3)jNa]+ and [(NaNO3)i(KNO3)jK]+ in the positive ion mode, where i + j = 1, 2, 3 … 10. Two‐component solutions of KNO3NaClO4 formed ions of the type [(KNO3)i(NaClO4)j(KClO4)k(NaNO3)lK]+ and [(KNO3)i(NaClO4)j(KClO4)k(NaNO3)lNa]+ in the positive ion mode, where i + j + k + l = 1, 2, 3 … 10. Similar clusters containing excess nitrate and perchlorate to provide the charge are formed in the negative ion mode. In each case, the maximum number of spectral lines for a cluster of size n can be calculated as the number of combinations of nth order (where n = i + j) of N different cation–anion pairs taken with replication and without regard for the ordering of the N cation–anion pairs. The actual number of lines observed may be reduced due to degeneracy of nominal m/z values for some ions. Copyright © 2006 John Wiley & Sons, Ltd.
Two methods for estimating aeroelastic damping of operational wind turbine modes from experiments
Authors: Hansen M. H. , Thomsen K. , Fuglsang P. , Knudsen T. 
Published: January 2006

AbstractThe theory and results of two experimental methods for estimating the modal damping of a wind turbine during operation are presented. Estimations of the aeroelastic damping of the operational turbine modes (including the effects of the aerodynamic forces) give a quantitative view of the stability characteristics of the turbine. In the first method the estimation of modal damping is based on the assumption that a turbine mode can be excited by a harmonic force at its natural frequency, whereby the decaying response after the end of excitation gives an estimate of the damping. Simulations and experiments show that turbine vibrations related to the first two tower bending modes can be excited by blade pitch and generator torque variations. However, the excited turbine vibrations are not pure modal vibrations and the estimated damping is therefore not the actual modal damping. The second method is based on stochastic subspace identification, where a linear model of the turbine is estimated alone from measured response signals by assuming that the ambient excitation from turbulence is random in time and space. Although the assumption is not satisfied, this operational modal analysis method can handle the deterministic excitation, and the modal frequencies and damping of the first tower and first edgewise whirling modes are extracted. Copyright © 2006 John Wiley & Sons, Ltd.
Spatial variation in abundance and catch composition of Cancer pagurus in Norwegian waters: biological reasoning and implications for assessment
Authors: Woll Astrid K. , van der Meeren Gro I. , Fossen Inge 
Published: January 2006

Abstract
The edible crab (Cancer pagurus), which is abundant along the Norwegian coast north to Troms County, has been exploited in Norway since the start of the 20th century. The main fishery is in Mid-Norway and Helgeland (63°–67°N), which together land 75% of the Norwegian catch. The fishery is regulated by season, minimum legal size, and the discarding of soft or ovigerous females. Catches have never been systematically described, so this study presents data collected over 4 years by a reference fleet of professional fishers reporting the catch from four standardized trial traps set among their ordinary traps. Catch rate, catch composition, and size distribution from Mid-Norway/Helgeland are compared with the smaller crab fishery in Rogaland (59°N) and with new crab grounds off Vesterålen (69°N). Local variations in size and sex composition between and within region seemed partly to be caused by differences between exposed and sheltered regions, and between heavily and newly exploited grounds. Size distribution is used as an indicator to determine a suitable sampling strategy. The most efficient survey design is seemingly the collection of relatively small samples from many boats to reduce the between-vessel component of variation. A suggested strategy to obtain an abundance index would be to collect daily catch rates from all commercial landings.
Using global genome approaches to address problems in cod mariculture1
Authors: Goetz Frederick W. , McCauley Linda , Goetz Giles W. , Norberg Birgitta 
Published: January 2006

AbstractA number of techniques, including expressed sequence tag (EST) analysis, serial analysis of gene expression, and micro-arrays, are available to study the global expression and regulation of genes. Many of these techniques are being used for intensively reared fish such as trout, salmon, and catfish to study genes involved in growth, reproduction, and health. In contrast, relatively little is known about the composition and regulation of transcriptomes in gadids. However, several bottlenecks in cod mariculture might benefit from the discovery and analysis of genes involved in reproduction, growth, and disease. As a result, we have begun EST analysis of genes in the cod ovary. Complimentary DNA (cDNA) libraries of cod ovaries taken from females at oocyte final maturation and ovulation have been constructed, and 1361 ESTs have been analysed. As expected, several oocyte-related genes were observed, including various zona pellucida egg membrane proteins. However, pivotal cell cycle regulators such as cyclins, genes involved in the regulation of apoptosis such as the Bcl-2-related ovarian killer protein, and hormone receptor components were also observed. Finally, a cDNA for a potential novel cod antifreeze protein was observed 12 times, suggesting the existence of a cod egg-specific antifreeze protein.
Escaping the Japanese Pyramid: The Association of Small and Medium Sized Enterprise Entrepreneurs (SME Doyukai), 1947–1999
Authors: Ibata-Arens Kathryn , Obayashi Hiromichi 
Published: January 2006

In Japanese society the pinnacle of economic and political power resides in Tokyo conglomerates and elite ministries: the Ministry of International Trade and Industry (MITI) and the Ministry of Finance. Until now, the story of this power arrangement has been told from the perspective of national bureaucrats and big business executives. The image projected to the outside world has been of cooperative, trust-based relational contracting with big business at the top “taking care” of its suppliers and subsidiaries below. The story from the bottom, however, is one of technology expropriation (of patentable technology) and monopsony squeeze (unilateral cost-down demands, for example). Firms unwilling to toe the line have been wholly excluded from access to the benefits reserved for those at the top of the pyramid, where one finds lucrative main bank financing, government support, and copious technological information.This article offers a historical narrative of the political struggle by independent-minded entrepreneurs in postwar Japan. Central to the struggle has been the challenge of building broad-based coalitions to avoid becoming embedded in these hierarchies while at the same time trying to obtain alternative sources of finance and technological know-how. The most successful example of such efforts is the Association of Small and Medium Size Enterprise Entrepreneurs (SME Doyukai). The SME Doyukai has somehow managed to remain completely independent from the state, while most other small business associations have not. This independence has not been free, and the association has gone through a number of institutional dilemmas as a result. We analyze these dilemmas over time and offer comparative lessons.
Estimating Subglottal Pressure using Incomplete Airflow Interruption
Authors: Jiang Jack , Leder Charles , Bichler Allison 
Published: January 2006

AbstractObjective/Hypothesis: Subglottal pressure (SGP) measurements are often used to assess laryngeal function; however, current methods to measure SGP are invasive or do not estimate SGP during phonation. The purpose of this study was to devise a noninvasive technique to estimate SGP without interrupting phonation.Study Design: This was a methodologic study designed to calculate the SGP of a human subject.Methods: A new technique to estimate SGP was developed in which subject airflow was partially interrupted by introducing two different impedances (pneumatic resistors) through a balloon valve controlled mouthpiece. The changes in airflow across varying impedances were used to estimate SGP in human subjects based on the predictable relationships between SGP, impedance, and airflow measurements. The technique was used to assess eight human subjects (age 19–30) phonating at intensities of 65, 72, and 80 dB.Results: The estimated SGP from the human subjects fell within a range (5.52–8.91 cm H20) that was consistent with previous studies. As other studies have found, SGP estimations significantly increased as subjects phonated at greater intensities (P < .01). Intrasubject standard deviations compared favorably with those from previous studies of complete airflow interruption, suggesting satisfactory reliability. The technique was validated using a mechanical “pseudolung” device (r2 = 0.9982).Conclusions: Incomplete airflow interruption offers the potential for useful clinical application to assess the severity of vocal pathology by estimating SGP during continuous phonation.
Management of T3N1 Glottic Carcinoma: Therapeutic Outcomes
Authors: Spector J Gershon , Sessions Donald G. , Lenox Jason , Simpson Joseph 
Published: January 2006

AbstractObjective: The aim of the current study was to determine the best total laryngectomy (TL) approach to the treatment of T3N1 glottic cancer, to study the impact of early nodal disease on stage III glottic cancers, and to describe the preliminary results in a group of patients recently treated for laryngeal preservation (LP).Methods: A retrospective study of Tumor Research Project data were performed on previously untreated patients with T3N1 glottic squamous cell carcinoma who were treated with curative intent by TL and neck dissection (ND) with or without adjuvant radiation therapy (TL +/− RT) from April 1, 1955 to October 8, 1999 at Washington University School of Medicine/Barnes Jewish Hospital. A preliminary analysis of a similar group of patients more recently treated for LP (1‐1‐2000 to 1‐1‐2005) is reported.Results: Forty‐two patients with T3N1 glottic carcinoma were treated with TL and ND (TL/ND‐16) and TL with ND and TL (TL/ND/RT‐26). The 5 year observed survival (OS) and disease‐specific survival (DSS) for TL/ND were similar at 62.5%. The 5 year OS and DSS for TL/ND/RT were 53.8% and 58.3%, respectively. There was no survival difference between the two methods. The overall local‐regional control rate was 73.9% (11/42 recurrences). The overall recurrence rate was 38%, with 7.1% recurrence at both the primary site and neck. Recurrence was not related to treatment method. The overall salvage rate (5 year DSS after retreatment) was 20% with 50% salvage for patients with neck recurrence. No patients with local recurrence were survivors. The incidence of second primary cancers was 6.8%. More recently, 26 similar patients were treated with LP techniques. Preliminary results showed a 3 year OS of 63.5% and DSS of 76.8%. Local‐regional control was 85.4%. LP was 88.5%.Conclusions: The two TL modalities had statistically similar results in terms of survival, recurrence, and complications. Decreased DSS was seen in older patients (>65 years) and in patients with involved resection margins, recurrent disease, and distant metastasis. Patients with T3N1 glottic cancer had an 8% decrease in DSS compared to patients with T3N0 disease. Previously patients with T3N1 disease have been treated with TL resulting in loss of natural voice in all patients. Preliminary results on 26 patients with T3N1 disease, treated between 2000 and 2005 with voice preservation intent, indicate that the OS, DSS, and local‐regional control rates were similar to the TL group, whereas 88.5% of patients maintained natural voice and natural breathing. Use of LP techniques should be the initial therapeutic approach for patients with T3N1 glottic cancer.
A de Novo PABPN1 Germline Mutation in a Patient with Oculopharyngeal Muscular Dystrophy
Authors: Gürtler Nicolas , Plasilova Martina , Podvinec Mihael , Boesch Nemya , Müller Hansjakob , Heinimann Karl 
Published: January 2006

AbstractBackground: Oculopharyngeal muscular dystrophy (OPMD) is a late‐onset autosomal dominantly inherited disorder characterized by dysphagia, ptosis, and proximal limb weakness and is caused by germline mutations (triplet repeat expansions) in the polyadenylate binding protein nuclear 1 (PABPN1) gene.Objective: To describe a 70‐year‐old female patient with OPMD on the clinical and molecular genetic level and to develop a rapid and efficient molecular genetic screening method to study large patient groups.Methods: Detailed family history and clinical assessment of the OPMD patient were followed by mutation analysis of the PABPN1 gene by direct DNA sequencing and by our newly developed method, fluorescent PABPN1 polymerase chain reaction (PCR) product (flPPP) method. A cohort of 50 healthy Swiss probands was screened using the flPPP to assess the frequency of the (GCG)7 allele in the Swiss population. Cricopharyngeal myotomy was performed as treatment for dysphagia.Results: A heterozygous (GCG)9 triplet repeat expansion in PABPN1 was identified. Since the family history proved to be negative, the mutation is likely to have occurred de novo. The frequency of the (GCG)7 allele among healthy Swiss controls amounted to 1%. The flPPP method showed a sensitivity and specificity of 100%. Two years after cricopharyngeal myotomy, the patient is still relieved of dysphagia.Conclusions: An otolaryngologist should include OPMD in the differential diagnosis of a patient presenting with dysphagia, as this symptom can be the first sign of the disease and family history can be negative. Molecular genetic testing represents a highly accurate and rapid way to confirm the clinical diagnosis of OPMD. Cricopharyngeal myotomy relieves the patient of dysphagia in the majority of cases.