bluuebunny/crossref_metadata_2025_split huggingface dataset:
Analysis of short‐term behavioral effects of dietary cholesterol supplementation in Smith–Lemli–Opitz syndrome
Authors: Tierney Elaine , Conley Sandra K. , Goodwin Halima , Porter Forbes D. 
Published: December 2009

AbstractSmith–Lemli–Opitz syndrome (SLOS) is an inborn error of cholesterol synthesis due to mutations of 7‐dehydrocholesterol reductase (DHCR7). DHCR7 catalyzes the reduction of 7‐dehydrocholesterol (7DHC) to yield cholesterol in the final step of cholesterol biosynthesis. Phenotypically patients with SLOS have multiple malformations, cognitive deficits, and behavioral difficulties. Impaired DHCR7 activity results in the accumulation of 7DHC and frequently decreased cholesterol in blood and tissues. Dietary cholesterol supplementation has become standard therapy for SLOS, and anecdotal reports suggest rapid, marked clinical improvement of behavior problems. Although reported in the literature, beneficial behavioral effects of dietary cholesterol supplementation have not been formally documented through a randomized clinical trial. To address this we initiated a double‐masked, placebo‐controlled, cross‐over trial to test the hypothesis that dietary cholesterol supplementation has rapid beneficial effects on behavior. Our primary outcome measure was the hyperactivity subscale of the Aberrant Behavior Checklist (ABC). Hyperactivity is a symptom that has been reported to respond rapidly to dietary cholesterol supplementation. Secondary outcome measures included the total ABC score and other ABC subscale scores. Ten subjects completed this study. Although the trial was done under conditions similar to those reported to induce marked behavioral changes in SLOS patients, we observed no differences between treatment and placebo phases. The results of this study call into question anecdotal reports supporting rapid behavioral benefits previously reported for dietary cholesterol supplementation in SLOS and underscore the need for a larger placebo‐controlled trial. © 2009 Wiley‐Liss, Inc.
Deletion of the CUL4B gene in a boy with mental retardation, minor facial anomalies, short stature, hypogonadism, and ataxia
Authors: Isidor Bertrand , Pichon Olivier , Baron Sabine , David Albert , Le Caignec Cédric 
Published: December 2009

AbstractThe CUL4B gene encodes a member of Cullin–RING ubiquitin ligase complex. Point mutations in CUL4B were identified recently in patients with syndromic X‐linked mental retardation (XLMR). Here, using oligoarray‐based comparative genomic hybridization (array CGH), we identified a de novo deletion of the CUL4B gene in a boy with syndromic mental retardation, minor facial anomalies, short stature, delayed puberty, hypogonadism, relative macrocephaly, gait ataxia, and pes cavus, all manifestations described previously in patients with CUL4B point mutations. Interestingly, our patient also presented with aortic valvular “dysplasia” and vertebral anomalies similar to those seen in Scheuermann disease, both of which may also be part of this syndrome. This report further suggests that point mutations and deletions of the CUL4B gene lead to a recognizable phenotype. The association of facial anomalies, short stature, hypogonadism, and gait ataxia in a mentally retarded boy should prompt molecular analyses of the CUL4B gene. © 2009 Wiley‐Liss, Inc.
Variation and context of yawns in captive chimpanzees (Pan troglodytes)
Authors: Vick Sarah‐Jane , Paukner Annika 
Published: December 2009

AbstractPrimate yawns are usually categorized according to context (e.g. as a threat, anxious, or rest yawn), but there has been little consideration of whether these yawns are best regarded as a unitary behavior that only differs with respect to the context in which it is observed. This study examined the context and precise morphology of yawns in a group of 11 captive chimpanzees. Focal video sampling was used to describe the morphology and intensity of 124 yawns using ChimpFACS, a system for coding facial movements. Two distinct forms of yawn were identified, a full yawn and a yawn which is modified by additional actions that reduce the mouth aperture. These modified yawns may indicate some degree of voluntary control over facial movement in chimpanzees and, consequently, multiple functions of yawning according to context. To assess context effects, mean activity levels (resting, locomotion, and grooming) and scratching rates were compared one minute before and after each yawn. Locomotion was significantly increased following both types of yawn, whereas scratching rates significantly increased following modified yawns but decreased following full yawns. In terms of individual differences, males did not yawn more than females, although male yawns were of higher intensity, both in the degree of mouth opening and in the amount of associated head movement. These data indicate that yawning is associated with a change in activity levels in chimpanzees, but only modified yawns may be related to increased arousal. Different types of yawn can therefore be differentiated at the morphological level as well as context level. Am. J. Primatol. 72:262–269, 2010. © 2009 Wiley‐Liss, Inc.
Chemoselectivity and the Curious Reactivity Preferences of Functional Groups
Authors: Afagh Nicholas A. , Yudin Andrei K. 
Published: December 2009

AbstractAchieving high levels of chemoselectivity has been the Achilles’ heel of chemical synthesis. The excitement generated by the successful realization of chemoselective strategies underscores the painstaking efforts to define a set of conditions conducive to selection among the available reaction pathways. We discuss in this Review various aspects of chemoselectivity that have been addressed in a range of synthetic methods over the past decade. We have focused on the proposed mechanistic basis of the reactions under consideration in an attempt to categorize them and highlight the key concepts that have been emerging on the basis of these studies. Our overview of recent advances in chemoselective processes suggests that significant progress has been made, but a lot of challenges lie ahead.
Bemessung eines Stützbauwerks nach Eurocode – Teil 1: Ständige Bemessungssituation
Authors: Suda Jürgen , Hofmann Robert , Strauss Alfred , Wendner Roman 
Published: December 2009

AbstractDieser Beitrag zeigt das Zusammenwirken des Eurocode 2 (EN 1992‐x) und des Eurocode 7 (EN 1997‐1) bei der Bemessung von Gewichtsmauern aus Beton und Stahlbeton anhand eines durchgerechneten Beispiels.Design of concrete retaining structures basing on Eurocode standards – Part 1: persistent design situation. This contribution shows the use of Eurocode 2 (EN 1992‐x) and Eurocode 7 (EN 1997‐1) for designing of concrete retaining structures on an example.
Prolonged absorption of antimony(V) by the oral route from non‐inclusion meglumine antimoniate–β‐cyclodextrin conjugates
Authors: Ribeiro Raul R. , Ferreira Weverson A. , Martins Patricia S. , Neto Rubens L. M. , Rocha Olguita G. F. , Le Moyec Laurence , Demicheli Cynthia , Frézard Frédéric 
Published: December 2009

AbstractThe orally active composition comprising meglumine antimoniate (MA) and β‐cyclodextrin (β‐CD) differs markedly from conventional drug–CD complexes, since it combines a water‐soluble drug and a hydrophilic CD. In order to obtain insights into the mechanism(s) responsible for the improved oral delivery of the drug, physicochemical and pharmacokinetic studies were carried out. The composition investigated here was prepared at a 7:1 antimony(Sb)/β‐CD molar ratio, a condition that improves its solubility in water and allows the oral administration of a high dose of Sb in large animals. It was characterized by circular dichroism, 1H‐NMR, ESI‐MS and photon correlation spectroscopy. Pharmacokinetic data were obtained in Beagle dogs after oral administration of the composition at 100 mg Sb/kg. 1H‐NMR and ESI‐MS data supported the formation of non‐inclusion complexes between MA and β‐CD. Sub‐micron assemblies were also evidenced that slowly dissociate and presumably release the MA drug, upon reconstitution of the composition in water. Pharmacokinetic studies of MA and MA/β‐CD in dogs showed a prolongation of the serum mean residence time of Sb from 4.1 to 6.8 h, upon complexation of MA with β‐CD. Evidence was also obtained that Sb remains essentially under the form of pentavalent Sb‐meglumine complex, following gastro‐intestinal absorption from the MA/β‐CD composition. In conclusion, the present data support the model that the sustained drug release property of 7:1 MA/β‐CD composition resulted in the prolongation of MA absorption by the oral route and, consequently, in the increase of the drug mean residence time in serum. Copyright © 2009 John Wiley & Sons, Ltd.
Der Zebrafisch als vielseitiges Modellsystem. Vom Zierfisch zum Forschungsobjekt
Authors: Schredelseker Johann , Krumschnabel Gerhard 
Published: December 2009

AbstractIn den vergangenen Jahren hat sich der Zebrafisch vom Zierfisch zum weitverbreiteten Modellsystem der biomedizinischen Forschung entwickelt. Er ist einfach zu halten, produziert in rascher Folge viele Nachkommen, entwickelt sich schnell und außerhalb des Muttertiers und ist zudem in den frühen Entwicklungsphasen transparent. Entsprechend wurde er zuerst vor allem zur unmittelbaren Beobachtung von Entwicklungsvorgängen genutzt. Da er aber auch relativ einfach genetisch manipulierbar ist, wurde er in Folge zur Herstellung transgener Linien verwendet, in denen Fremd‐DNA exprimiert und die Bedeutung des jeweiligen Genprodukts für die Biologie des Tieres untersucht werden kann. Außerdem kann er durch Zufalls‐Mutagenese zur unvoreingenommenen Entdeckung krankheitsrelevanter Gene verwendet werden, zum Screening biologisch wirksamer Substanzen für die Entwicklung von Medikamenten und zunehmend auch als Modell in der Krebsforschung.
Liebe bei Tupajas. Warum sind die Weibchen bei der Partnerwahl so wählerisch?
Authors: von Holst Dietrich 
Published: December 2009

AbstractTupajas (Tupaia belangeri) leben in der Natur paarweise in Territorien. Erstaunlicherweise führt das Zusammensetzen eines männlichen und weiblichen Tupajas im Labor nur in knapp 25% aller Fälle zu einer harmonischen Paarbeziehung, die sich von Beginn an in freundlichem Verhalten zwischen den Partnern, einer Verbesserung ihres Gesundheitszustandes und einer erfolgreichen Fortpflanzung äußert; in den meisten Fällen werden jedoch alle Kontaktversuche der Männchen von den Weibchen abgewehrt, was zu mehr oder minder starken Stressreaktionen bei beiden Tieren führt. Entscheidend für die Akzeptanz eines Männchens durch das Weibchen ist sein Geruch beziehungsweise die dadurch vermittelten immungenetischen Informationen.
Von Neuronen zu Netzwerken. Mathematische Gehirnmodelle
Authors: Grah Gunnar , Rotter Stefan 
Published: December 2009

AbstractObwohl unser Verständnis des Nervensystems große Fortschritte macht, stellen Netzwerke aus Milliarden von Neuronen die Neurobiologie vor eine praktisch unlösbare Aufgabe: Die Aktivität eines Gehirns möglichst vollständig zu erfassen und das Beobachtete detailliert zu verstehen. Die “Computational Neuroscience” versucht Brücken zwischen den Konzepten der Teildisziplinen zu schlagen. Die mathematische Beschreibung von Nervenzellen und neuronalen Netzwerken, sowie die Simulation dieser Systeme in Form von Computermodellen, erlaubt Phänomene zu ergründen, die in biologischen Gehirnen nur unter größten Schwierigkeiten messbar sind. Jüngste Studien konnten unter anderem zeigen, dass erregungsabhängige Veränderungen der elektrischen Leitfähigkeit in Neuronen ein Netzwerk davor bewahren, dass räumlich begrenzte Erregung sich aufschaukelt und als Folge die Aktivität im gesamten Netzwerk zum Erliegen bringt. Dieselbe Eigenschaft führt außerdem dazu, dass ein Netzwerk auch ohne äußere Anregung aktiv bleiben kann – eine wichtige Grundeigenschaft von Gehirnen, deren neuronale Funktionsmechanismen bis heute weitgehend unverstanden sind.
Wie werden Bakterien optimal gelagert?
Authors: Schrödel Andrea 
Published: December 2009

AbstractFür die Stammhaltung von Bakterien werden diese in vielen Laboratorien periodisch auf frische Nährböden überimpft und im Kühlschrank gelagert. Dadurch sind die Bakterien zwar schnell verfügbar, doch es bestehen bei diese Methode einige Gefahren, wie die Infektion mit Luftkeimen, eine spontane Mutation der Produktionsstämme, Verwechslungen, oder gar eine Überwachsung der hergestellten Mutante durch den Wildtyp (der oft schneller wächst) und ist daher für Hochleistungsstämme eher ungeeignet. Wie konserviert man einen mühsam hergestellten Bakterienklon auf Dauer?
Adolf Remane – Zoologe und Entdecker des Mesopsammons
Authors: Storch Volker 
Published: December 2009

AbstractDer Kieler Zoologe Adolf Remane wird als eine Persönlichkeit in Erinnerung bleiben, die das Gesamtgebiet der Zoologie beherrschte, einen weltweiten neuen Lebensraum mit einer Fülle bis dahin unbekannter Organismen entdeckte, wissenschaftliche Beiträge von den niederen Metazoen bis zu Primaten verfasste, Grundlagen der vergleichenden Biologie schuf und Anregungen für die Soziobiologie vermittelte.
Von Sing‐ und Dachsammern
Authors: Irsch Wilhelm 
Published: December 2009

AbstractDie auf dem nordamerikanischen Kontinent verbreiteten Singammern (Melospiza melodia) erlernen ihren arttypischen Gesang von ihrem zweiten Lebensmonat an und orientieren sich dabei vor allem am Gesang der Nachbarn. Die Männchen sind wahre Gesangsakrobaten. Ihre Gesänge bestehen aus wiederholten Sequenzen, in die rasch vorgetragene, allein stehende Passagen und Pfiffe eingestreut sind. Dachsammern (Zonotrichia leucophrys) aus der Region um San Francisco singen hingegen immer eintöniger – eine Reaktion auf den zunehmenden Verkehrslärm.
Using life cycle approaches to enhance the value of corporate environmental disclosures
Authors: Kaenzig Josef , Friot Damien , Saadé Myriam , Margni Manuele , Jolliet Olivier 
Published: December 2009

AbstractAs the focus of environmental policy and management shifts from cleaner production at the process level towards greener products as a whole, stakeholders ask for transparency throughout the entire value chain. This article assesses the comprehensiveness and the value of currently reported quantitative environmental disclosures of 97 listed companies from the automotive, banking, pharmaceutical and electronic hardware sectors. Findings indicate that quantitative environmental disclosures have many limitations, including incompleteness and inconsistency regarding corporate activities and sites, and limited internal data coherence. For many sectors, corporate disclosures only cover a very small share of the total environmental burden of products. A stepwise procedure is proposed to verify and improve the quality and completeness of reporting using life cycle approaches. We present simple data quality tests, and we introduce the concept of the environmental influence matrix, which provides a solid basis for the identification and prioritization of key performance indicators and areas of action. Copyright © 2009 John Wiley & Sons, Ltd and ERP Environment.
Feed development for fed‐batch CHO production process by semisteady state analysis
Authors: Khattak Sarwat F. , Xing Zizhuo , Kenty Brian , Koyrakh Inna , Li Zheng Jian 
Published: December 2009

AbstractSemisteady state cultures are useful for studying cell physiology and facilitating media development. Two semisteady states with a viable cell density of 5.5 million cells/mL were obtained in CHO cell cultures and compared with a fed‐batch mode control. In the first semisteady state, the culture was maintained at 5 mM glucose and 0.5 mM glutamine. The second condition had threefold higher concentrations of both nutrients, which led to a 10% increase in lactate production, a 78% increase in ammonia production, and a 30% reduction in cell growth rate. The differences between the two semisteady states indicate that maintaining relatively low levels of glucose and glutamine can reduce the production of lactate and ammonia. Specific amino acid production and consumption indicated further metabolic differences between the two semisteady states and fed‐batch mode. The results from this experiment shed light in the feeding strategy for a fed‐batch process and feed medium enhancement. The fed‐batch process utilizes a feeding strategy whereby the feed added was based on glucose levels in the bioreactor. To evaluate if a fixed feed strategy would improve robustness and process consistency, two alternative feeding strategies were implemented. A constant volume feed of 30% or 40% of the initial culture volume fed over the course of cell culture was evaluated. The results indicate that a constant volumetric‐based feed can be more beneficial than a glucose‐based feeding strategy. This study demonstrated the applicability of analyzing CHO cultures in semisteady state for feed enhancement and continuous process improvement. © 2009 American Institute of Chemical Engineers Biotechnol. Prog., 2010
Haloacetamidine‐Based Inactivators of Protein Arginine Deiminase 4 (PAD4): Evidence that General Acid Catalysis Promotes Efficient Inactivation
Authors: Knuckley Bryan , Causey Corey P. , Pellechia Perry J. , Cook Paul F. , Thompson Paul R. 
Published: December 2009

AbstractDysregulated protein arginine deiminase (PAD) activity, particularly PAD4, has been suggested to play a role in the onset and progression of numerous human diseases, including rheumatoid arthritis (RA). Given the potential role of PAD4 in RA, we set out to develop inhibitors/inactivators that could be used to modulate PAD activity and disease progression. This effort led to the discovery of two mechanism‐based inactivators, denoted F‐ and Cl‐amidine, that inactivate PAD4 by the covalent modification of an active‐site cysteine that is critical for catalysis. To gain further insights into the mechanism of inactivation by these compounds, the effect of pH on the rates of inactivation was determined. These results, combined with the results of solvent isotope effect and proton inventory studies, strongly suggest that the inactivation of PAD4 by F‐ and Cl‐amidine proceeds by a multistep mechanism that involves the protonation and stabilization of the tetrahedral intermediate formed upon nucleophilic attack by the active‐site cysteine, that is, Cys645. Stabilization of this intermediate would help to drive the halide‐displacement reaction, which results in the formation of a three‐membered sulfonium ring that ultimately collapses to form the inactivated enzyme. This finding—that protonation of the tetrahedral intermediate is important for enzyme inactivation—also suggests that, during catalysis, protonation of the analogous intermediate is required for efficient substrate turnover.
A New Highly Selective and Sensitive Assay for Fluorescence Imaging of .OH in Living Cells: Effectively Avoiding the Interference of Peroxynitrite
Authors: Li Ping , Xie Ting , Duan Xia , Yu Fabiao , Wang Xu , Tang Bo 
Published: December 2009

AbstractA new nonredox fluorescent probe to realize the imaging of hydroxyl radicals (.OH) in living cells was designed and synthesized. The structure comprised the fluorescent dye boron dipyrromethene (BDP) and a 2,2,6,6‐tetramethyl‐1‐piperidinoxyl (TEMPO) unit. This probe could rapidly respond to .OH with a detection limit of 18 pM, and it possessed superior photostability and pH insensitivity. Other reactive oxygen species (ROS) and relevant intracellular components did not interfere. In particular, the important problem of ONOO− interference was efficiently avoided. An MTT assay proved that the probe was not very cytotoxic. The probe could penetrate into intact cell membranes to selectively detect intracellular .OH without causing cellular damage in living mice macrophages, normal human liver cells. and human hepatoma cells. These advantageous characteristics make the fluorescent probe potentially useful as a new candidate to detect .OH in broad biosystems.
Synthesis of a Metal–Organic Framework Material, Iron Terephthalate, by Ultrasound, Microwave, and Conventional Electric Heating: A Kinetic Study
Authors: Haque Enamul , Khan Nazmul Abedin , Park Jung Hwa , Jhung Sung Hwa 
Published: December 2009

AbstractA metal–organic framework material named MIL‐53(Fe), iron terephthalate, has been synthesized sovothermally at a relatively low temperature by not only conventional electric (CE) heating, but also by irradiation under ultrasound (US) and microwave (MW) conditions to gain an understanding of the accelerated syntheses induced by US and MW. The kinetics for nucleation and crystal growth were analyzed by measuring the crystallinity of MIL‐53(Fe) under various conditions. The nucleation and crystal growth rates were estimated from crystallization curves of the change in crystallinity with reaction time. The activation energies and pre‐exponential factors were calculated from Arrhenius plots. It was confirmed that the rate of crystallization (both nucleation and crystal growth) decreases in the order US>MW≫CE, and that the accelerated syntheses under US and MW conditions are due to increased pre‐exponential factors rather than decreased activation energies. It is suggested that physical effects such as hot spots are more important than chemical effects in the accelerated syntheses induced by US and MW irradiation. The syntheses were also conducted in two steps to understand quantitatively the acceleration induced by MW and it was found that the acceleration in crystal growth is more important than the acceleration in nucleation, even though both processes are accelerated by MW irradiation.
Efficient spatiotemporal analysis of the flagellar waveform of Chlamydomonas reinhardtii
Authors: Bayly P. V. , Lewis B. L. , Kemp P. S. , Pless R. B. , Dutcher S. K. 
Published: December 2009

AbstractThe 9 + 2 axoneme is a microtubule‐based machine that powers the oscillatory beating of cilia and flagella. Its highly regulated movement is essential for the normal function of many organs; ciliopathies cause congenital defects, chronic respiratory tract infections and infertility. We present an efficient method to obtain a quantitative description of flagellar motion, with high spatial and temporal resolution, from high speed video recording of bright field images. This highly automated technique provides the shape, shear angle, curvature, and bend propagation speeds along the length of the flagellum, with ∼200 temporal samples per beat. We compared the waveforms of uniflagellated wild‐type and ida3 mutant cells, which lack the I1 inner dynein complex. Video images were captured at 350 fps. Rigid‐body motion was eliminated by fast Fourier transform (FFT)‐based registration, and the Cartesian (x–y) coordinates of points on the flagellum were identified. These x–y “point clouds” were embedded in two data dimensions using Isomap, a nonlinear dimension reduction method, and sorted by phase in the flagellar cycle. A smooth surface was fitted to the sorted point clouds, which provides high‐resolution estimates of shear angle and curvature. Wild‐type and ida3 cells exhibit large differences in shear amplitude, but similar maximum and minimum curvature values. In ida3 cells, the reverse bend begins earlier and travels more slowly relative to the principal bend, than in wild‐type cells. The regulation of flagellar movement must involve I1 dynein in a manner consistent with these results. © 2009 Wiley‐Liss, Inc.