bluuebunny/crossref_metadata_2025_split huggingface dataset:
First chemical synthesis of a scorpion α‐toxin affecting sodium channels: The Aah I toxin of Androctonus australis hector
Authors: M'Barek Sarrah , Fajloun Ziad , Cestèle Sandrine , Devaux Christiane , Mansuelle Pascal , Mosbah Amor , Jouirou Besma , Mantegazza Massimo , van Rietschoten Jurphaas , Ayeb Mohamed El , Rochat Hervé , Sabatier Jean‐Marc , Sampieri Franc̨ois 
Published: June 2004

AbstractAah I is a 63‐residue α‐toxin isolated from the venom of the Buthidae scorpion Androctonus australis hector, which is considered to be the most dangerous species. We report here the first chemical synthesis of Aah I by the solid‐phase method, using a Fmoc strategy. The synthetic toxin I (sAah I) was renatured in DMSO‐Tris buffer, purified and subjected to thorough analysis and comparison with the natural toxin. The sAah I showed physico‐chemical (CD spectrum, molecular mass, HPLC elution), biochemical (amino‐acid composition, sequence), immunochemical and pharmacological properties similar to those of the natural toxin. The synthetic toxin was recognized by a conformation‐dependent monoclonal anti‐Aah I antibody, with an IC50 value close to that for the natural toxin. Following intracerebroventricular injection, the synthetic and the natural toxins were similarly lethal to mice. In voltage‐clamp experiments, Nav1.2 sodium channel inactivation was inhibited by the application of sAah I or of the natural toxin in a similar way. This work describes a simple protocol for the chemical synthesis of a scorpion α‐toxin, making it possible to produce structural analogues in time. Copyright © 2004 European Peptide Society and John Wiley & Sons, Ltd.
Solid phase peptide synthesis on epoxy‐bearing methacrylate monoliths
Authors: Vlakh E. , Novikov A. , Vlasov G. , Tennikova T. 
Published: June 2004

AbstractMonoliths based on a copolymer of glycidyl methacrylate (GMA) and ethylene dimethacrylate (EDMA) can be used directly as sorbents for affinity chromatography after solid phase peptide synthesis. The quality of the synthesized products, the amount of grown peptides on a support and the reproducibility of the process must be considered. A determination of the quantity of the introducing β‐Ala (and, consequently, the total amount of synthesized peptide) was carried out. Three peptides complementary to recombinant tissue plasminogen activator (t‐PA) have been synthesized using Fmoc‐chemistry on GMA‐EDMA disks. The peptidyl ligands were analysed by amino acid analysis, ES‐MS and HPLC methods.The affinity binding parameters were obtained from frontal elution data. The results were compared with those established for GMA‐EDMA affinity sorbents formed by the immobilization of the same but separately synthesized and purified ligands. The immobilization on GMA‐EDMA disks was realized using a one‐step reaction between the amino groups of the synthetic ligand and the original epoxy groups of monolithic material. The affinity constants found for two kinds of sorbent did not vary significantly. Finally, the directly obtained affinity sorbents were tested for t‐PA separation from a cellular supernatant. Copyright © 2004 European Peptide Society and John Wiley & Sons, Ltd.
Optimal Design of the Adaptive Sample Size and Sampling Interval np Control Chart
Authors: Wu Zhang , Luo Hua 
Published: June 2004

AbstractRecent research has shown that the adaptive control charts are quicker than the traditional static charts in detecting process shifts. This paper develops the algorithm for the optimization designs of the adaptive np control charts for monitoring the process fraction non‐conforming p. It includes the variable sample size chart, the variable sampling interval chart, and the variable sample size and sampling interval chart. The performance of the adaptive np charts is measured by the average time to signal under the steady‐state mode, which allows the shift in p to occur at any time, even during the sampling inspection. By studying the performance of the adaptive np charts systematically, it is found that they do improve effectiveness significantly, especially for detecting small or moderate process shifts. Copyright © 2004 John Wiley & Sons, Ltd.
Multivariate Extensions to Cumulative Sum Control Charts
Authors: Runger George C. , Testik Murat C. 
Published: June 2004

AbstractThere are several multivariate extensions to the venerable cumulative sum (CUSUM) chart. This work provides a comparison of the advantages and disadvantages of each, as well as performance evaluations and a description of their interrelationships. A new derivation is also provided. Extensive simulation results that include initial and steady‐state conditions are presented. Geometric descriptions are used, and names are proposed based on these geometric characteristics. As several of the key steps in the development of a multivariate extension to a CUSUM also appear in the construction of a two‐sided CUSUM, some approaches to a two‐sided CUSUM are also summarized. Copyright © 2004 John Wiley & Sons, Ltd.
A Genetic Algorithm Hybrid for Constructing Optimal Response Surface Designs
Authors: Drain David , Carlyle W. Matthew , Montgomery Douglas C. , Borror Connie , Anderson‐Cook Christine 
Published: June 2004

AbstractHybrid heuristic optimization methods can discover efficient experiment designs in situations where traditional designs cannot be applied, exchange methods are ineffective, and simple heuristics like simulated annealing fail to find good solutions. One such heuristic hybrid is GASA (genetic algorithm–simulated annealing), developed to take advantage of the exploratory power of the genetic algorithm, while utilizing the local optimum exploitive properties of simulated annealing. The successful application of this method is demonstrated in a difficult design problem with multiple optimization criteria in an irregularly shaped design region. Copyright © 2004 John Wiley & Sons, Ltd.
A New Method for Constructing Confidence Intervals for the Index Cpm
Authors: Perakis Michael , Xekalaki Evdokia 
Published: June 2004

AbstractIn the statistical literature on the study of the capability of processes through the use of indices, Cpm appears to have been one of the most widely used capability indices and its estimation has attracted much interest. In this article, a new method for constructing approximate confidence intervals or lower confidence limits for this index is suggested. The method is based on an approximation of the non‐central chi‐square distribution, which was proposed by Pearson. Its coverage appears to be more satisfactory compared with that achieved by any of the two most widely used methods that were proposed by Boyles, in situations where one is interested in assessing a lower confidence limit for Cpm. This is supported by the results of an extensive simulation study. Copyright © 2004 John Wiley & Sons, Ltd.
Accuracy of the electrostatic theorem for high‐quality Slater and Gaussian basis sets
Authors: Fernández Rico J. , López R. , Ema I. , Ramírez G. 
Published: June 2004

AbstractThe fulfillment of the Hellmann–Feynman electrostatic theorem is examined for the sequences of cc‐pVxZ and cc‐pCVxZ Gaussian basis sets as well as for the VBx and CVBx basis sets of Slater‐type orbitals. The difference between the energy gradient and electrostatic forces is large in small Gaussian basis sets of the two types, but decreases quickly as the basis sets improve. In VBx Slater basis sets these differences are small but the improvement is irregular, whereas in CVBx basis sets the fulfillment of the electrostatic theorem is very satisfactory. For the high‐quality basis sets (cc‐pV5Z, cc‐pCVQZ, cc‐pCV5Z, CVB2, and CVB3) the energy gradient can be replaced by the electrostatic force in most practical applications. © 2004 Wiley Periodicals, Inc. Int J Quantum Chem, 2004
Electronic band structure evolution as a carbon sheet rolls up to a SWCNT and a SWCNT closes to a torus, by using extended SSH model
Authors: Sünel N. , Özsoy O. 
Published: June 2004

AbstractWe relate the electronic band structure to parameters characterizing geometry and, by varying these parameters, we obtain the continuous evolution of the electronic band structure of a carbon sheet to that of a SWCNT and to that of a torus, in the framework of the extended SSH model. As well, we evaluate the bond distortions. The calculated electronic properties clearly confirm the literature on other tight‐binding approximations. Finally, we break the constraint into two parts and reexamine the electronic band structures and the bond distortions. © 2004 Wiley Periodicals, Inc. Int J Quantum Chem, 2004
Hepatocyte transplantation for inborn errors of metabolism
Authors: Burlina A. B. 
Published: June 2004

AbstractSummary: Liver transplantation for inborn errors of metabolism has proved effective in some (mostly liver‐associated) inborn errors of metabolism. Significant morbidity and mortality rates have been extensively reported due to disease recurrence or to complications of the immunosuppressive regimen. On the basis of clinical trials in animals as well as in humans, the use of isolated hepatocytes offers a unique opportunity for treating inborn errors of metabolism. The state of art of the technique applied to this field is reviewed here and related practical problems are examined. No final conclusions can be drawn, but further insight into the use of alternative sources of cells, including stem/progenitor cells associated with cryobiology and immunological research, will offer new opportunities for cell therapy for inborn errors of metabolism in the near future.
The genetics and pathophysiology of diabetes mellitus type II
Authors: Jenkins A. B. , Campbell L. V. 
Published: June 2004

AbstractSummary: Type II diabetes is a common, complex and heterogeneous group of disorders of growing public health concern. Paradoxically, rare monogenic forms of diabetes mellitus have been the most informative regarding diabetes pathophysiology to date. We discuss disappointing results of genetic approaches thus far, emphasizing the genetic heterogeneity underlying the common phenotypic endpoint of elevated blood glucose level and the phenotypic misclassification in large studies resulting from this admixture and from the obligatory use of epidemiological or clinical surrogate measures. We suggest that novel approaches that take explicit account of the phenotypic, environmental and genetic complexities of type II diabetes are needed and discuss some principles that might underlie such approaches.
After the genome—the phenome?
Authors: Scriver C. R. 
Published: June 2004

AbstractSummary:What next? The Human Genome Project signifies complexity rather than simplification in the relationship between genotype and phenotype. Genotypes are embedded in genomes. Individuality in phenotypes is embedded in components ofthe phenome(transcriptome, metabolome, proteome, etc.). The phenome, its layers, and its nodes, links and networks, require elucidation; there is a need for a Human Phenome Project (Freimer and Sabatti 2003). Biology has largely been a reductive science in the recent past; integrative biology lies ahead. Clinician‐scientists (including human biochemical geneticists) will be recognized as key participants in the ‘medical’ Phenome Project as it reveals components of individuality, and their contributions, in simple or combinatorial fashion, to Mendelian and complex traits; better ways to treat ‘genetic disease’ will be by‐products of the project.Although the Word is common to all, most men live as if each had a private wisdom of his own.Herakleitos
Enzyme replacement and enhancement therapies for lysosomal diseases
Authors: Desnick R. J. 
Published: June 2004

AbstractSummary: Although first suggested by de Duve in 1964, enzyme replacement therapy (ERT) for lysosomal storage diseases did not become a reality until the early 1990s when its safety and effectiveness were demonstrated in type 1 Gaucher disease. Today, ERT is a reality for Gaucher disease, Fabry disease and mucopolysaccharidosis type I (MPS I), and clinical trials with recombinant human enzymes are ongoing in Pompe disease, MPS II and MPS VI, and are about to begin in Neimann–Pick B disease. In addition to ERT, enzyme enhancement therapy (EET) offers a novel therapeutic strategy to increase the residual function of mutant proteins. EET employs small molecules as 'pharmacological chaperones' to rescue misfolded and/or unstable mutant enzymes or proteins that have residual function. EET also offers the possibility of treating neurodegenerative lysosomal disorders since these small therapeutic molecules may cross the blood–brain barrier. The current status of ERT and the prospects for EET for lysosomal storage diseases are reviewed.
Treatment of lysosomal storage disorders: Cell therapy and gene therapy
Authors: Eto Y. , Shen J. ‐S. , Meng X. ‐L. , Ohashi T. 
Published: June 2004

AbstractSummary: Most lysosomal storage diseases have central nervous system (CNS) involvement. No effective treatment is available at present. We investigated the usefulness of brain‐directed gene therapy and cell therapy using mouse models of lysosomal storage diseases. For gene therapy to the CNS, a recombinant adenovirus encoding β‐galactocerebrosidase gene was injected into the cerebral ventricle of neonatal twitcher mice, a murine model of Krabbe disease. Improvements in neurological symptoms and a prolonged lifespan were observed. Brain activity of β‐galactocerebrosidase was increased significantly and the concentration of a cytotoxic metabolite, psychosine, was decreased. Pathological observations of the brain were also improved in treated twitcher mice. For cell therapy to the CNS, a neural stem cell line derived from human fetal brain was genetically engineered to overexpress β‐glucuronidase and transplanted into the cerebral ventricles of neonatal MPS VII mice, a model of β‐glucuronidase deficiency. Transplanted human neural stem cells were found to integrate and migrate in the host brain and to produce large amounts of β‐glucuronidase. Brain contents of the substrate of β‐glucuronidase were reduced and widespread clearing of lysosomal storage was observed in treated MPS VII mice. These data suggest that brain‐directed gene/cell therapy may be useful in the treatment of neurological alterations in lysosomal storage diseases.
Congenital disorders of glycosylation (CDG): Update and new developments
Authors: Jaeken J. 
Published: June 2004

AbstractSummary: After a brief overview on CDG, this workshop concentrated on the experience with (mostly) known CDG in a European country (the Czech Republic) and on the Australasian experience, on recent developments regarding congenital muscular dystrophies due to O‐mannosylglycan assembly defects, and on new presentations of CDG. It was concluded that we are still at the beginning of ‘explosive’ research on CDG and that we need to apply new and known technologies to the diagnosis, understanding of pathophysiology, and treatment of CDG.
Glycine encephalopathy (nonketotic hyperglycinaemia): Review and update
Authors: Applegarth D. A. , Toone J. R. 
Published: June 2004

AbstractSummary: This article summarizes data and issues covered in the workshop on Glycine Encephalopathy using headings that cover important topics in our present knowledge of this disease.Presenters were: Dr Ada Hamosh (Baltimore); Dr Johan L. K. Van Hove (Leuven); Dr Christine Vianey‐Saban (Lyon); Dr Shigeo Kure (Sendai); Dr A. Ichinohe (Sendai); Jennifer Toone (Vancouver); Dr Stanley Korman (Jerusalem).
How does a carabid predator find aggregations of slugs in the field? Electroantennograms and behavioural assays suggest chemical cues
Authors: McKemey A.R. , Glen D.M. , Symondson W.O.C. 
Published: June 2004

AbstractField studies suggest that the generalist carabid predator and scavengerPterostichus melanariusIlliger aggregates to patches with a higher density of slug prey. The mechanisms behind such aggregation are unknown. Experiments were conducted to test the hypothesis that they are responding to chemical cues. Electroantennograms (EAGs) showed no response byP. melanariusantennae to live, nematode-infected, injured or freshly killed slugs, but a strong response to dead slugs that had been allowed to decay for up to 48 h. Such a response would enable the beetles to find carrion in the field and may also, as dead prey are likely to be spatially correlated with live ones, provide a mechanism by whichP. melanariusfinds patches with a higher density of live slugs. Subsequent video analyses ofP. melanariusresponses to patches of slug mucus within arenas showed that beetles with intact antennae could detect these patches because they spent more time, and moved greater distances, within them and increased their rate of turning. Thus, at close range,P. melanariusused their antennae to detect slug mucus and hence, by implication, live slugs. The apparent contradiction between EAG data and behavioural experiments is discussed. Together these result confirm thatP. melanariusdoes respond to chemical cues from its slug prey in ways that could lead to aggregation in areas of higher slug density in the field.
Diet breadth and its relationship with genetic diversity and differentiation: the case of southern beech aphids (Hemiptera: Aphididae)
Authors: Gaete-Eastman C. , Figueroa C.C. , Olivares-Donoso R. , Niemeyer H.M. , Ramírez C.C. 
Published: June 2004

AbstractHerbivorous insect species with narrow diet breadth are expected to be more prone to genetic differentiation than insect species with a wider diet breadth. However, a generalist can behave as a local specialist if a single host-plant species is locally available, while a specialist can eventually behave as a generalist if its preferred host is not available. These problems can be addressed by comparing closely related species differing in diet breadth with overlapping distributions of insect and host populations. In this work, diet breadth, genetic diversity and population differentiation of congeneric aphid species from southern beech forests in Chile were compared. While at the species level no major differences in genetic diversity were found, a general trend towards higher genetic diversity as diet breadth increased was apparent. The aphid species with wider diet breadth, Neuquenaphis edwardsi (Laing), showed the highest genetic diversity, while the specialist Neuquenaphis staryi Quednau & Remaudière showed the lowest. These differences were less distinct when the comparisons were made in the same locality and over the same host. Comparison of allopatric populations indicates that genetic differentiation was higher for the specialists, Neuquenaphis similis Hille Ris Lambers and N. staryi, than for the generalist N. edwardsi. Over the same host at different locations, genetic differentiation among populations of N. edwardsi was higher than among populations of N. similis. The results support the assumption that specialists should show more pronounced genetic structuring than generalists, although the geographical distribution of host plants may be playing an important role.
Staphylinidae and Carabidae overwintering in wheat and sown wildflower areas of different age
Authors: Frank T. , Reichhart B. 
Published: June 2004

AbstractSpecies richness and abundance of staphylinid and carabid beetles overwintering in winter wheat fields and 1- to 3-year-old wildflower areas were investigated during 2000/2001 on 16 study sites in Switzerland. Abundance and species richness of overwintering staphylinids significantly increased with successional age of the wildflower areas and were always higher in older wildflower areas than in winter wheat. A similar but less distinct pattern was observed for the abundance and species richness of carabid beetles. The influence of habitat parameters (vegetation cover, fine sand content, organic matter, pH, soil pore volume, surrounding landscape structure, habitat area) on the staphylinid and carabid assemblages based on the number of individuals per species and site was analysed using canonical correspondence analysis. Vegetation cover was the most significant parameter significantly characterizing both staphylinid and carabid assemblages. The amount of vegetation cover explained 15.7% of the variance, fine sand content accounted for 13.3% and surrounding landscape structure for 10.9% of the variance in the staphylinid assemblage. In the carabid assemblage, vegetation cover was the only significant factor, explaining 24.7% of the variance. This study showed for the first time that the significance of wildflower areas as a reservoir for hibernation for generalist predatory beetles increases with progressing successional age.
An updated list of the ticks of Ghana and an assessment of the distribution of the ticks of Ghanaian wild mammals in different vegetation zones
Authors: Ntiamoa-Baidu Y. , Carr-Saunders C. , Matthews B.E. , Preston P.M. , Walker A.R. 
Published: June 2004

AbstractTwenty one species of ticks belonging to five genera of the family Ixodidae (Order Acari, sub-order Ixodida) – Amblyomma, Haemaphysalis, Hyalomma, Ixodes and Rhipicephalus (including the sub-genus Rhipicephalus (Boophilus)) – were collected from 1260 mammals, representing 29 species, 14 families and 6 orders, in four vegetation zones in Ghana during the period 1971–1978. Four other species were collected from humans in 1977. In all, eight species appeared to be new records for Ghana: Amblyomma tholloni Neumann; Dermacentor circumguttatus Neumann; Haemaphysalis houyi Nuttall & Warburton; Ixodes loveridgei Arthur; Ixodes oldi Nuttall; Ixodes vanidicus Schultze; Rhipicephalus complanatus Neumann; Rhipicephalus cuspidatus Neumann. The updated list of tick species in Ghana given here includes 41 species of ixodid ticks and four species of argasid ticks. Most species have been found in neighbouring regions of West Africa but 56 of the 121 different combinations of ixodid tick species and host species found in the collection described here have not apparently been reported before. The new combinations recorded here bring the total number of different combinations of ixodid tick species and mammalian host species now reported in Ghana to 151. The tick species found on wild mammals in Ghana mostly differed from those reported from domestic stock by other authors. The data showed that different tick species occurred in different vegetation zones and that most species displayed a pronounced preference for certain groups of related host species. Some tick species were found in the savanna feeding mainly on large bovids and/or suids; others were found in forests feeding mainly on small bovids, large rodents or small carnivores.