bluuebunny/crossref_metadata_2025_split huggingface dataset:
A novel mutation in LMBRD1 causes the cblF defect of vitamin B12 metabolism in a Turkish patient
Authors: Gailus Susann , Suormala Terttu , Malerczyk‐Aktas Ayse Gül , Toliat Mohammad R. , Wittkampf Tanja , Stucki Martin , Nürnberg Peter , Fowler Brian , Hennermann Julia B. , Rutsch Frank 
Published: February 2010

AbstractIn the cblF defect of vitamin B12 (cobalamin) metabolism, cobalamin is trapped in lysosomes. Consequently, cobalamin coenzyme synthesis is blocked, and cofactors for methionine synthase and methylmalonyl‐coenzyme A (CoA) mutase are deficient. We recently identified LMBRD1 as the causative gene located on chromosome 6q13 and showed that 18 out of 24 alleles in unrelated patients carried the deletion c.1056delG (p.L352fsX18) (Rutsch et al. (Nat Genet 41:234–239, 2009). LMBRD1 encodes the lysosomal membrane protein LMBD1, which presumably facilitates lysosomal cobalamin export. Our patient is the second child of consanguineous Turkish parents. He presented on the second day of life with cerebral seizures due to intraventricular hemorrhage. Plasma homocysteine and urinary methylmalonic acid levels were elevated, and serum cobalamin level was decreased. Synthesis of both cobalamin coenzymes was deficient in cultured skin fibroblasts. The cblF defect was confirmed by somatic complementation analysis. Sequencing of LMBRD1 revealed the novel deletion c.1405delG (p.D469fsX38) on both alleles. Real‐time polymerase chain reaction (PCR) revealed reduced messenger RNA (mRNA) levels in patient fibroblasts compared with controls. Transfection of patient fibroblasts with the LMBD1 wild‐type complement DNA (cDNA) rescued coenzyme synthesis and function, confirming this new deletion as an additional cause of the cblF defect. This case adds to the spectrum of clinical presentations and mutations of this rare disorder of lysosomal transport.
Does relationship learning lead to relationship value? A cross‐national supply chain investigation
Authors: Cheung Mee‐Shew , Myers Matthew B. , Mentzer John T. 
Published: February 2010

AbstractIn global business‐to‐business markets, shared resources between buyers and suppliers often result in competitive advantages and enhanced relationships between firms. Unfortunately, there is a paucity of research regarding learning capabilities between business partners in a cross‐border setting. This study takes the approach to integrate customer value literature into interorganizational learning theory and adopts the often‐neglected theoretical perspective of transaction value by contextualizing inter‐firm collaboration in terms of relationship learning and value co‐creation viewed by both the buyers and sellers in one single study. Through the development of a conceptual framework that examines how global environmental and inter‐organizational conditions influence learning capabilities, the study investigates how relationship learning influences relationship value for both supplying and buying firms. Using a survey of 126 cross‐border dyads in the industrial chemical, packaging, consumer durable, and apparel industries, the authors show how relationship learning is valued by both buyers and suppliers, and how it is critical when viewing the “supplier as a customer.” The results indicate the strategic nature of relationship learning in maintaining cross‐border business‐to‐business relationships. Simultaneously, the findings provide evidence that cultural distance is not a significant influence on the firm's propensity to share knowledge with its global partners. It helps advance our understanding of the significance of cultural‐pollination in the era of globalization.
The Formation of International Organizations and India: A Historical Study
Authors: ANAND R. P. 
Published: February 2010

AbstractAs the clash of aspirations increased among European countries, a European ‘civil war’ started in 1914, which engulfed the whole world. With all the terrible destruction and loss of life, it was felt that an international organization must be established to avert war in future. At the Paris Peace Conference in 1919, the British government succeeded in gaining separate representation for its dominions, including India. This created a rather anomalous situation, since a dependency of a foreign power, a colony which could not control its internal affairs, was accepted as a sovereign state by an international treaty. Europe had hardly recovered from the First World War in the late 1920s when it drifted towards a second holocaust in 1939. India became a founding member of the United Nations in 1945, even though it was still under British rule, participating in the historic founding conference. But Indian national public opinion was neither very hopeful nor enthusiastic about the conference on the new international organization. Not only India, which was not even independent at that time, but Asian countries as such played a very small and insignificant role in the formulation of the UN Charter.
INTERNATIONAL CRIMINAL COURTS ROUND-UP
Authors: Guellali Amna , Rojo Enrique Carnero 
Published: February 2010

AbstractThe International Criminal Courts Round-Up reports on the most interesting judgements and decisions rendered by international(ized) criminal courts and tribunals during the reporting period. In addition, important developments within the various organs of the courts are highlighted, such as the appointment of new judges and significant amendments to the procedural rules of the courts. The Round-Up presently covers issues concerning the International Criminal Court, the International Criminal Tribunal for the former Yugoslavia, the International Criminal Tribunal for Rwanda and the Special Court for Sierra Leone.
SOME CONTRARIAN REFLECTIONS ON ADVERTISING
Authors: Machan Tibor R. 
Published: February 2010

Among business ethics teachers, as reflected in their books and papers, advertising is deemed anything but honorable. Quite the opposite. This is mainly because so many business ethicists are convinced that altruism is the proper ethics for people to practice and, of course, advertising is far from altruistic. The following will be a presentation of a position that finds advertising ethical but also rejects altruism as the proper ethics by which human beings should live.
The Relationship between Physical Activity and Body Mass Index: Issues in Model Specification
Authors: Tiruneh Gizachew 
Published: February 2010

Abstract
Objective:
To investigate the best statistical models that describe the effect of physical activity on BMI.*Design*: Cross-sectional analyses of physical activity and BMI data. *Subjects*: 107 obese, overweight, and healthy college students (mean duration of physical activity for the normal, overweight, and obese students: 89, 59, and 24 months, respectively; mean BMI for the normal, overweight, and obese students: 21.61, 27.07, and 35.54 kg/m2, respectively).*Measurements*: Inverse linear, inverse logarithmic, and inverse logistics models were used to analyze survey data for physical activity (measured by both frequency and duration of exercise) and BMI. Gender, age, and physical intensity variables were also statistically controlled. *Results*: Coefficients of determination, r-squared, showed the inverse logarithmic model is more accurate in describing the effect of physical activity on BMI than is the inverse linear model. The inverse logistic method also showed physical activity affects BMI.

Conclusions:
Although the inverse logarithmic method can be used in some cases, the inverse logistic model seems to be theoretically and empirically best suited in describing the relationship between physical activity and body weight.
Electric field enhanced hydrogen storage on polarizable materials substrates
Authors: Zhou J. , Wang Q. , Sun Q. , Jena P. , Chen X. S. 
Published: February 2010

Using density functional theory, we show that an applied electric field can substantially improve the hydrogen storage properties of polarizable substrates. This new concept is demonstrated by adsorbing a layer of hydrogen molecules on a number of nanomaterials. When one layer of H
2
molecules is adsorbed on a BN sheet, the binding energy per H
2
molecule increases from 0.03 eV/H
2
in the field-free case to 0.14 eV/H
2
in the presence of an electric field of 0.045 a.u. The corresponding gravimetric density of 7.5 wt% is consistent with the 6 wt% system target set by Department of Energy for 2010. The strength of the electric field can be reduced if the substrate is more polarizable. For example, a hydrogen adsorption energy of 0.14 eV/H
2
can be achieved by applying an electric field of 0.03 a.u. on an AlN substrate, 0.006 a.u. on a silsesquioxane molecule, and 0.007 a.u. on a silsesquioxane sheet. Thus, application of an electric field to a polarizable substrate provides a novel way to store hydrogen; once the applied electric field is removed, the stored H
2
molecules can be easily released, thus making storage reversible with fast kinetics. In addition, we show that materials with rich low-coordinated nonmetal anions are highly polarizable and can serve as a guide in the design of new hydrogen storage materials.
The transition state for folding of an outer membrane protein
Authors: Huysmans Gerard H. M. , Baldwin Stephen A. , Brockwell David J. , Radford Sheena E. 
Published: February 2010

Inspired by the seminal work of Anfinsen, investigations of the folding of small water-soluble proteins have culminated in detailed insights into how these molecules attain and stabilize their native folds. In contrast, despite their overwhelming importance in biology, progress in understanding the folding and stability of membrane proteins remains relatively limited. Here we use mutational analysis to describe the transition state involved in the reversible folding of the β-barrel membrane protein PhoPQ-activated gene P (PagP) from a highly disordered state in 10 M urea to a native protein embedded in a lipid bilayer. Analysis of the equilibrium stability and unfolding kinetics of 19 variants that span all eight β-strands of this 163-residue protein revealed that the transition-state structure is a highly polarized, partly formed β-barrel. The results provide unique and detailed insights into the transition-state structure for β-barrel membrane protein folding into a lipid bilayer and are consistent with a model for outer membrane protein folding via a tilted insertion mechanism.
Functional regions of the N‐terminal domain of the antiterminator RfaH
Authors: Belogurov Georgiy A. , Sevostyanova Anastasia , Svetlov Vladimir , Artsimovitch Irina 
Published: February 2010

SummaryRfaH is a bacterial elongation factor that increases expression of distal genes in several long, horizontally acquired operons. RfaH is recruited to the transcription complex during RNA chain elongation through specific interactions with a DNA element called ops. Following recruitment, RfaH remains bound to RNA polymerase (RNAP) and acts as an antiterminator by reducing RNAP pausing and termination at some factor‐independent and Rho‐dependent signals. RfaH consists of two domains connected by a flexible linker. The N‐terminal RfaH domain (RfaHN) recognizes the ops element, binds to the RNAP and reduces pausing and termination in vitro. Functional analysis of single substitutions in this domain reported here suggests that three separate RfaHN regions mediate these functions. We propose that a polar patch on one side of RfaHN interacts with the non‐template DNA strand during recruitment, whereas a hydrophobic surface on the opposite side of RfaHN remains bound to the β′ subunit clamp helices domain throughout transcription of the entire operon. The third region is apparently dispensable for RfaH binding to the transcription complex but is required for the antitermination modification of RNAP.
Chronic alcoholism associated with diabetes impairs erectile function in rats
Authors: Lizarte Fermino S. , Morgueti Marcelo , Tirapelli Carlos R. , Claudino Mario A. , Evora Paulo R. B. , Novais Paulo C. , Tirapelli Daniela P. C. , Celotto Andrea C. , Capellini Verena K. , Celini Fabia P. M. , Tucci Jr Silvio , Cologna Adauto J. , Antunes Edson , Martins Antonio C. P. , Tirapelli Luis F. 
Published: February 2010

OBJECTIVETo investigate the effects of chronic ethanol consumption and diabetes on nitric oxide (NO)‐mediated relaxation of cavernosal smooth muscle (CSM).MATERIAL AND METHODSMale Wistar rats were divided into four groups: control, isocaloric, diabetic and ethanol‐diabetic. The CSMs were mounted in organ chambers for measurement of isometric tension. Contraction of the strips was induced by electrical field stimulation (EFS, 1–32 Hz) and phenylephrine. We also evaluated the effect of ethanol consumption on the relaxation induced by acetylcholine (ACh; 0.01–1000 µmol/L), sodium nitroprusside (SNP, 0.01–1000 µmol/L) or EFS (1–32 Hz) in strips pre‐contracted with phenylephrine (10 µmol/L). Immunoexpression of endothelial NO synthase (eNOS) and inducible NOS (iNOS) was also accessed.RESULTSThe endothelium‐dependent relaxation induced by ACh was decreased in CSM from ethanol‐diabetic rats when compared with the controls, with a mean (sem) of 21 (4) vs 37 (2)%. Similarly, the potency and maximal responses induced by SNP were reduced in the ethanol‐diabetic [3.97 (0.38) and 85 (1)%, respectively] and diabetic groups [3.78 (0.56) and 81 (2)%, respectively] when compared with the controls [5.3 (0.22) and 90 (3)%, respectively] and isocaloric [5.3 (0.19) and 92 (1)%, respectively] groups. Noradrenergic nerve‐mediated contractions of CSM in response to EFS were increased in rats from ethanol‐diabetic and diabetic groups when compared with the control and isocaloric groups. Conversely, there were no differences in EFS‐induced relaxation among the groups. The immunostaining assays showed overexpression of eNOS and iNOS in the CSM from diabetic and ethanol‐diabetic rats when compared with the control and isocaloric rats.CONCLUSIONThere was an impairment of relaxation of CSM from ethanol‐diabetic and diabetic rats that involved a decrease in the NO‐cyclic guanosine monophosphate signalling pathway by endothelium‐dependent mechanisms accompanied by a change in the CSM contractile sensitivity.
Research Productivity and Social Capital in Australian Higher Education
Authors: Salaran Mohammad 
Published: February 2010

AbstractThis study investigates the role of social capital in raising research productivity in academic institutions. Social capital as a strategic resource embedded in social relationships can be utilised towards decreasing pressures from external environmental conditions, such as the global financial crisis. A survey was sent to academic staff in five universities in Victoria, to collect data regarding their frequency of communications and research productivity. The findings indicated that there is a significant and positive correlation between social interactions and research productivity. Regression analysis demonstrated that social interactions as an independent variable predict research productivity of academics.
Developing a contemporary conceptualization for consumer sciences in higher education in the UK
Authors: Bailey Sue 
Published: February 2010

AbstractConsumer sciences and consumer studies as a recent interdisciplinary degree area in higher education in the UK has had limited published research either on the subject heritage or its disciplinary identity. Since the major subject review was undertaken by the Council for National Academic Awards 17 years ago, the research investigation aimed to study how consumer studies and home economics had moved to consumer sciences and consumer studies. The research has therefore involved a survey over a 15‐year period of development and changes of institutions and degrees and a content analysis of programme outlines and national benchmarking to reveal changing trends in how the subject has been conceptualized. The comparison used data from 1992, 1999, 2003 and 2007 to map degree programme and institutional changes. External and internal political and economic factors influenced subject growth, decline and development. It was found that the number of degree programme titles had remained relatively stable, with still a balance between BA and BSc degrees but that this disguised many changes in institutions and degree titles.The content analysis of trends was undertaken, comparing 1992, 1999, 2003 and 2007. Subject benchmarking statements introduced in 2002, currently being revised in 2009, were compared with programme content themes in 2003 and 2007. The content analysis showed that the overall approach or principles, areas of study, context, perspectives and processes for degree programmes at the undergraduate level had developed noticeably over the last 15 years and had been clarified by benchmarking guidelines. Themes of behavioural change, consumer ethics, consumer services, skills and education, sustainability and well‐being had developed with applied contexts depending on programme orientation.A model has been proposed to illustrate the current conceptualization of the subject in the UK at degree level, and Chenoweth's definition of consumer sciences and consumer studies has been redefined for the UK. It is recommended that there should be more theoretical reflection on the interpretation and development of the subject.
Genetic variability of Old Portuguese bread wheat cultivars assayed by IRAP and REMAP markers
Authors: Carvalho A. , Guedes‐Pinto H. , Martins‐Lopes P. , Lima‐Brito J. 
Published: February 2010

Retrotransposons (RTNs) constitute informative molecular markers for plant species as a result of their ability of integrating into a multitude of loci throughout the genome and thereby generating insertional polymorphisms between individuals. Inter‐retrotransposon amplified polymorphisms (IRAPs) and the retrotransposon‐microsatellite amplified polymorphisms (REMAPs) are marker systems based on long terminal repeats (LTRs) RTNs, developed for plants, that have been widely used for evolution, genetic diversity, DNA fingerprinting of cultivars and varieties, genetic mapping linkage and for detection of genetic rearrangements induced by polyploidisation. In the present study, we aimed to analyse the genetic variability among 48 Old Portuguese bread wheat cultivars using both IRAP and REMAP markers. Five IRAP and six REMAP primer combinations were used. IRAP produced 103 polymorphic fragments in a total of 113 bands. On average, 22.6 bands were amplified per IRAP primer combination. The bands ranged in size from 250 to 5000 bp. The REMAP primer combinations allowed the amplification of 53 bands, 51 of them polymorphic. An average of 8.8 REMAP bands was scored per primer combination. The REMAP bands ranged from 250 to 3000 bp.Both marker systems presented high percentages of polymorphism. However, IRAP markers were suitable for detecting genetic variability at the individual level and did not differentiate higher taxa. The REMAP maker system allowed the clustering by botanical variety and identified most of the homonym bread wheat cultivars.
Mitochondrial proline oxidation is affected by hyperosmotic stress in durum wheat seedlings
Authors: Soccio M. , Laus M.N. , Spera G.P. , Trono D. , Pompa M. , Flagella Z. , Pastore D. 
Published: February 2010

The important role of plant mitochondria in the adaptation to environmental stresses at subcellular level has recently emerged. In particular, an important mitochondrial mechanism involved in the resistance to environmental stresses is the inhibition of proline oxidation. In order to study this physiological mechanism, we used both washed and purified durum wheat (Triticum durum) mitochondria (DWM) isolated from early seedlings germinated in two different NaCl solutions leading to either moderate or severe damage to growth. To assess the contribution of the osmotic component of stress, a parallel investigation was performed using hyperosmotic mannitol solutions.Comparison of the oxygen uptake rate in the course of proline oxidation, with that of the malate plus glutamate substrate pair and that of succinate showed that an early inhibition of proline oxidation occurs under stress. The drop of the proline‐dependent oxygen uptake rate was as a result of a heavy inhibition of proline dehydrogenase (ProDH); on the other hand, malate plus glutamate‐dependent and succinate‐dependent oxidations were less inhibited, being the maintenance of oxygen uptake rate not dependent on alternative oxidase (AOX) pathway; in fact, DWM‐AOX activity did not show any increase under our experimental stress conditions. The selective inhibition of proline oxidation should be considered a mitochondrial adaptation to stress rather than damage to mitochondrial oxidative properties. This result was achieved by means of a novel approach based on the comparison between oxygen uptake rates of washed and purified organelles.
School District Democracy: School Board Voting and School Performance
Authors: WEBBER DAVID J. 
Published: February 2010

In line with social capital theory, citizen involvement in local education policy making should affect education performance. Specifically, because voting turnout and candidate competition in school district elections are accountability mechanisms and reflections of a district's social capital, these characteristics of school board elections should affect how schools perform. Using official Missouri election records and school district data, this article examines the effect of district social capital (i.e., voting turnout and candidate competition), demographics, and school resources on school performance (i.e., standardized test scores and graduation rates). Mixed support is found for social capital theory, with voting turnout significantly affecting standardized test scores, though not graduation rates, and candidate competition influencing graduation rates, though in an unexpected direction. This study expands our understanding of the factors affecting school performance and informs the discussion of education reform.En línea con la teoría del capital social, la participación de los ciudadanos en la hechura de las políticas de educación local debería afectar el rendimiento escolar. Específicamente, porque la participación en la votación y el grado de competencia entre candidatos en las elecciones en los distritos escolares son mecanismos de rendición de cuentas y un reflejo del capital social del distrito, estas características de las elecciones del consejo escolar deberían afectar el desempeño de las escuelas. Utilizando registros oficiales de las elecciones en Missouri e información de los distritos escolares, este artículo examina el efecto del capital social del distrito (i.e., participación en la votación y el grado de competencia entre candidatos), la demografía, y los recursos escolares en el rendimiento escolar (i.e., los resultados estandarizados de las pruebas y las tasas de graduación). Se encuentra un apoyo mixto para la teoría del capital social donde la participación en la votación afecta considerablemente los resultados estandarizados de las pruebas, aunque no a las tasas de graduación, y en una dirección inesperada, el grado de competencia entre candidatos influye a las tasas de graduación. Este articulo amplia nuestro entendimiento de los factores que afectan el rendimiento escolar y fortalece la discusión sobre la reforma educativa.