bluuebunny/crossref_metadata_2025 huggingface dataset:
International Psychology
Authors: Stevens Michael J. , Zeinoun Pia
Published: March 2013

International psychology is a branch of psychology whose definition defies categorical classification, such as by area of interest (e.g., clinical psychology) or by methodology (e.g., research methods). International psychology not only has manifold dimensions, as reflected in its mission and scope, but also is dynamic given its co‐variation with the history of the discipline and its relevance to a constantly changing world.
Worldviews
Authors: Chakkarath Pradeep
Published: March 2013

The term
worldview
– which is a loan translation from the German
Weltanschauung
or
Weltsicht
– refers to an elaborate cognitive framework or belief/orientation system that provides societies and individuals with a means of understanding their environment and ordering their lives in accordance with various key assumptions that are widely shared by the members of their ingroups.
Recent Developments at Foster + Partners' Specialist Modelling Group
Authors: De Kestelier Xavier
Published: March 2013

Established 15 years ago under the leadership of Hugh Whitehead, the Specialist Modelling Group (SMG) at Foster + Partners has an unrivalled reputation internationally as an in‐house research group. It provides Fosters with expertise in computation, geometry and fabrication, as well as in environmental analysis and simulation. Xavier De Kestelier, co‐head of SMG and partner at Foster + Partners, describes how the group has developed and expanded over the last few years, growing the range of its specialist knowledge that informs projects as diverse as the YachtPlus Boat Fleet (2009), Kuwait International Airport (2009‐) and Masdar Institute of Science and Technology (2010).
Symmetry As Geometry: Kuwait International Airport
Authors: Josefsson Kristoffer
Published: March 2013

Computer‐aided design (CAD) software has often been associated with the limitations of Euclidean geometry. Kristoffer Josefsson, a mathematician and specialist geometer employed in the Specialist Modelling Group (SMG) at Foster + Partners, explains how the symmetry‐encoded representation undertaken for Kuwait International Airport enabled the group to extend CAD tools for non‐Euclidean use.
Mathematical Ensemble: Molteni Arc Table
Authors: Hon Jethro
Published: March 2013

Jethro Hon is an architect working in the Specialist Modelling Group (SMG) at Foster + Partners, specialising in computational methods with a particular focus on the optimisation of complex geometries, fabrication and building performance. Here he describes how a table designed for Italian furniture‐manufacturer Molteni, inspired by tensile fabric structures, was generated through the group's experimentation with simulation software.
Integrated Computational Design: National Bank of Kuwait Headquarters
Authors: Popovska Dusanka
Published: March 2013

Foster + Partners' design for the National Bank of Kuwait Headquarters is for an environmentally responsive building and a complex geometry. Dusanka Popovska, a design system analyst in the Specialist Modelling Group (SMG) at Fosters, explains how the group was introduced at an early stage in the bank's design process to develop a parametric model that was capable of integrating various performance parameters while continuing to investigate geometrical solutions.
Spatial Computing for the New Organic
Authors: Derix Christian , Izaki Åsmund
Published: March 2013

In the last few years, parametric design has predominated in architectural research and practice. Christian Derix and Åsmund Izaki of the Computational Design and Research (CDR) group at Aedas|R&D explain how the research and design unit, based at Aedas in London, has pursued an alternative course by shifting the focus to behavioural and perceptive occupant simulation of spatial configurations. A human‐centric approach, it draws its inspiration from earlier British pioneers in spatial computation, as well as the designers of the Organic Architecture movement of the early 20th century.
Interoperability in Sports Design
Authors: Hines David
Published: March 2013

David Hines leads the parametric and advanced geometry group at Populous, the global design practice renowned for its expertise in sports facility design. Here he describes how Populous and Buro Happold created a shared model environment for the Aviva Stadium in Dublin (2010) , enabling a smooth parametric‐to‐BIM‐to fabrication process. A watershed initiative, it provided the prerequisite stepping stone for developing a parametric bowl tool for designing stadia.
From Model Thinking to Process Design
Authors: Knippers Jan
Published: March 2013

The introduction of computational design processes and particularly of computer‐aided fabrication methods recast roles across the design team. As Jan Knippers, founding partner of Knippers Helbig Advanced Engineering and Head of the Institute of Building Structures and Structural Design (ITKE) at the University of Stuttgart explains, it offers structural engineers a unique opportunity: the potential to break through the barriers of conventional model thinking (thinking in discrete typologies) and to embrace process design and new forms of interaction.
Navigating the Computational Turn
Authors: van Berkel Ben
Published: March 2013

UNStudio has developed its own practice model for connecting colleagues and collaborators and sharing accumulated knowledge and techniques: the ‘Knowledge Platform’. Ben van Berkel with members from the UNStudio Smart Parameter Platform describe three projects at very different scales ‐ the Burnham Pavilion in Chicago (2009), Raffles City in Hangzhou, China (2011) and Arnhem Central (2012) ‐ that encapsulate UNStudio's working methods and approach to computational processes.
Embedding Intelligence: Architecture and Computation at Grimshaw, NY
Authors: Edwards Seth
Published: March 2013

Research and development at Grimshaw is concentrated in two groups: Design Technology (DT) and Industrial Design (ID). Seth Edwards from Grimshaw's DT group explains how these dual hubs provide an essential resource for the practice, offering expertise in complex geometry, rapid prototyping and construction. He illustrates three projects, originating in the New York office, that exemplify this approach.
Linking Structure and Parametric Geometry
Authors: Preisinger Clemens
Published: March 2013

The Karamba plug‐in developed by Clemens Preisinger in collaboration with Bollinger + Grohmann Engineers has been developed to predict the behaviour of structures under external loads. Intended to be used by architects rather than being solely confined to an engineering setting, it enables a seamless flow of data between structural and geometric models. Preisinger here describes the program's evolution and application.
Design Ecosystems: Customising the Architectural Design Environment with Software Plug‐ins
Authors: Davis Daniel , Peters Brady
Published: March 2013

As a preface to this section dedicated to the rise of plug‐ins, Daniel Davis and Guest‐Editor Brady Peters describe the shift in architectural culture that has seen the adoption of scripting interfaces ‐ once the preserve of CAD specialists ‐ spread to designers using code to create geometry and find form. They explain how this defines an entirely new landscape in which ‘cathedrals’ (monolithic applications) are challenged by ‘bazaars’ (generative‐modelling editors) populated by animal‐named plug‐ins, where architects become the formulators rather than just the end users of programs.
Molten‐Salt‐Assisted Self‐Assembly (MASA)‐Synthesis of Mesoporous Metal Titanate‐Titania, Metal Sulfide‐Titania, and Metal Selenide‐Titania Thin Films
Authors: Karakaya Cüneyt , Türker Yurdanur , Dag Ömer
Published: March 2013

New synthetic strategies are needed for the assembly of porous metal titanates and metal chalcogenite‐titania thin films for various energy applications. Here, a new synthetic approach is introduced in which two solvents and two surfactants are used. Both surfactants are necessary to accommodate the desired amount of salt species in the hydrophilic domains of the mesophase. The process is called a molten‐salt‐assisted self‐assembly (MASA) because the salt species are in the molten phase and act as a solvent to assemble the ingredients into a mesostructure and they react with titania to form mesoporous metal titanates during the annealing step. The mesoporous metal titanate (meso‐Zn2TiO4 and meso‐CdTiO3) thin films are reacted under H2S or H2Se gas at room temperature to yield high quality transparent mesoporous metal chalcogenides. The H2Se reaction produces rutile and brookite titania phases together with nanocrystalline metal selenides and H2S reaction of meso‐CdTiO3 yields nanocrystalline anatase and CdS in the spatially confined pore walls. Two different metal salts (zinc nitrate hexahydrate and cadmium nitrate tetrahydrate) are tested to demonstrate the generality of the new assembly process. The meso‐TiO2‐CdSe film shows photoactivity under sunlight.
Nematic Liquid Crystals Embedded in Cubic Microlattices: Memory Effects and Bistable Pixels
Authors: Serra Francesca , Eaton Shane Michael , Cerbino Roberto , Buscaglia Marco , Cerullo Giulio , Osellame Roberto , Bellini Tommaso
Published: March 2013

The confinement of liquid crystals in geometries with frustrating boundary conditions gives rise to nontrivial effects such as bistability and memory. It is shown that large memory effects arise when nematic liquid crystals are embedded in cubic micrometer‐sized scaffolds made by two‐photon polymerization. The electric field alignment of the liquid crystals inside the porous medium is maintained when the applied field is above a threshold (approximately 2 V per micrometer of cell thickness). The onset of the memory is an on/off type process for each individual pore of the scaffold, and the memory typically starts emerging in one region of the structure and then propagates. The global memory effects in porous structures with controlled geometry are enhanced with respect to the case of random porous structures. This work is a proof of the “memory from topology” principle, which was previously suggested by computer simulations. These new materials can pave the way to new types of bistable displays.
Creation of Ghost Illusions Using Wave Dynamics in Metamaterials
Authors: Jiang Wei Xiang , Qiu Cheng‐Wei , Han Tiancheng , Zhang Shuang , Cui Tie Jun
Published: March 2013

The creation of wave‐dynamic illusion functionality is of great interest to various scientific communities because it can potentially transform an actual perception into the pre‐controlled perception, thus empowering unprecedented applications in the advanced‐material science, camouflage, cloaking, optical and/or microwave cognition, and defense security. By using the space transformation theory and engineering capability of metamaterials, a functional “ghost” illusion device, which is capable of creating multiple virtual ghost images of the original object's position under the illumination of electromagnetic waves, is proposed and realized. The scattering signature of the object is thus ghosted and perceived as multiple ghost targets with different geometries and compositions. The ghost‐illusion material, which is being inhomogeneous and anisotropic, is realized using thousands of varying unit cells working at non‐resonance. The experimental demonstration of the ghost illusion validates the theory of scattering metamorphosis and opens a novel avenue to the wave‐dynamic illusion, cognitive deception, manipulate strange light (or matter) behaviors, and design novel optical and microwave devices.
An Electrochemically Actuated MEMS Device for Individualized Drug Delivery: an In Vitro Study
Authors: Song Peiyi , Tng Danny Jian Hang , Hu Rui , Lin Guimiao , Meng Ellis , Yong Ken‐Tye
Published: March 2013

Individualized disease treatment is a promising branch for future medicine. In this work, we introduce an implantable microelectromechanical system (MEMS) based drug delivery device for programmable drug delivery. An in vitro study on cancer cell treatment has been conducted to demonstrate a proof‐of‐concept that the engineered device is suitable for individualized disease treatment. This is the first study to demonstrate that MEMS drug delivery devices can influence the outcome of cancer drug treatment through the use of individualized disease treatment regimes, where the strategy for drug dosages is tailored according to different individuals. The presented device is electrochemically actuated through a diaphragm membrane and made of polydimethylsiloxane (PDMS) for biocompatibility using simple and cost‐effective microfabrication techniques. Individualized disease treatment was investigated using the in vitro programmed delivery of a chemotherapy drug, doxorubicin, to pancreatic cancer cell cultures. Cultured cell colonies of two pancreatic cancer cell lines (Panc‐1 and MiaPaCa‐2) were treated with three programmed schedules and monitored for 7 days. The result shows that the colony growth has been successfully inhibited for both cell lines among all the three treatment schedules. Also, the different observations between the two cell lines under different schedules reveal that MiaPaCa‐2 cells are more sensitive to the drug applied. These results demonstrate that further development on the device will provide a promising novel platform for individualized disease treatment in future medicine as well as for automatic in vitro assays in drug development industry.
A Light‐Driven Anti‐Cancer Dual‐Therapeutic Cassette Enhances Solid Tumour Regression
Authors: Kim A Ra , Shin Seung Won , Cho Seung‐Woo , Lee Joo Young , Kim Dong‐Ik , Um Soong Ho
Published: March 2013

The majority of anticancer therapeutics have failed to control the target cancers. Thus, new rational design concepts are critical. In most of the biological reactions, a cascade pathway is used to activate appropriate responses. In the cascade pathway, a small signal derived from neighboring environments can be amplified and it further triggers overwhelming and specialized responses. It can be applied to achieve powerful therapeutic effects for novel drug design strategies. Inspired by this concept, we design a preferential dual anti‐cancer therapeutic cassette composed of (i) DNA/RNA nanostructures as both anticancer containers and target ligands and (ii) a gold nanocrystal as localized heat inducers. We demonstrate that this multi‐modular platform is superior to conventional cancer medications in that it had higher drug loading efficiency, tunable drug release, and intrinsic serum stability characteristics. Both doxorubicin chemotherapy and thermal ablation exert a powerful synergistic killing effect that resulted in prostate cancer regression both in vitro and in vivo. We speculate that our novel anti‐cancer drug system can be adapted to effectively destroy many different types of solid cancers.
Enhanced Osteogenesis by a Biomimic Pseudo‐Periosteum‐Involved Tissue Engineering Strategy
Authors: Shi Xuetao , Chen Song , Zhao Yihua , Lai Chen , Wu Hongkai
Published: March 2013

Elaborating a bone replacement using tissue‐engineering strategies for bone repair seems to be a promising remedy. However, previous platforms are limited in constructing three‐dimensional porous scaffolds and neglected the critical importance of periosteum (a pivotal source of osteogenic cells for bone regeneration). We report here an innovative method using the periosteum as a template to replicate its exquisite morphologies onto the surfaces of biomaterials. The precise topographic cues (grooved micropatterns) on the surface of collagen membrane inherited from the periosteum effectively directed cell alignment as the way of natural periosteum. Besides, we placed the stem‐cell and endothelial‐cell‐laden collagen membrane (pseudo‐periosteum) onto a three‐dimensional porous scaffold. The pseudo‐periosteum‐covered scaffolds showed remarkable osteogenesis when compared with the pseudo‐periosteum‐free scaffolds, indicating the significant importance of pseudo‐periosteum on bone regeneration. This study gives a novel concept for the construction of bone tissue engineering scaffold and may provide new insight for periosteum research.
Prospects and Challenges of Graphene in Biomedical Applications
Authors: Bitounis Dimitrios , Ali‐Boucetta Hanene , Hong Byung Hee , Min Dal‐Hee , Kostarelos Kostas
Published: March 2013

Graphene materials have entered a phase of maturity in their development that is characterized by their explorative utilization in various types of applications and fields from electronics to biomedicine. Herein, we describe the recent advances made with graphene‐related materials in the biomedical field and the challenges facing these exciting new tools both in terms of biological activity and toxicological profiling in vitro and in vivo. Graphene materials today have mainly been explored as components of biosensors and for construction of matrices in tissue engineering. Their antimicrobial activity and their capacity to act as drug delivery platforms have also been reported, however, not as coherently. This report will attempt to offer some perspective as to which areas of biomedical applications can expect graphene‐related materials to constitute a tool offering improved functionality and previously unavailable options.
Evaluation of Ion‐Exchange Kinetic Parameters Using Poly‐o‐anisidine and Poly‐o‐toluidine Sn(IV) Tungstate Nanocomposite Cation Exchangers
Authors: Khan Asif Ali , Shaheen Shakeeba
Published: March 2013

Organic–inorganic nanocomposites poly‐o‐anisidine and poly‐o‐toluidine Sn(IV) tungstate were chemically synthesized by mixing organic polymers that is, o‐anisidine and o‐toluidine with inorganic gels of Sn(IV) tungstate in different mixing ratios. Ion‐exchange kinetics for few divalent metal ions was evaluated by particle diffusion‐controlled ion‐exchange phenomenon at four different temperatures. The particle diffusion mechanism was confirmed by linear τ (dimensionless time parameter) versus t (time) plots. The exchange processes were controlled by the diffusion of ions within the exchanger particle for the systems under studies. Some physical parameters such as self‐diffusion coefficient (), energy of activation (Ea), and entropy of activation (ΔS°) were evaluated under conditions favoring a particle diffusion‐controlled mechanism. © 2013 Wiley Periodicals, Inc. Adv Polym Technol 2013, 32, 21346; View this article online at wileyonlinelibrary.com. DOI 10.1002/adv.21346
Investigation of Driving Forces for Charge Extraction in Organic Solar Cells: Transient Photocurrent Measurements on Solar Cells Showing S‐Shaped Current–Voltage Characteristics
Authors: Tress Wolfgang , Corvers Steef , Leo Karl , Riede Moritz
Published: March 2013

The role of drift and diffusion as driving forces for charge carrier extraction in flat heterojunction organic solar cells is examined at the example of devices showing intentional S‐shaped current–voltage (J‐V) characteristics. Since these kinks are related to energy barriers causing a redistribution of the electric field and charge carrier density gradients, they are suitable for studying the limits of charge extraction. The dynamics of this redistribution process are experimentally monitored via transient photocurrents, where the current response on square pulses of light is measured in the μs to ms regime. In combination with drift‐diffusion simulation data, we demonstrate a pile‐up of charge carriers at extraction barriers and a high contribution of diffusion to photocurrent in the case of injection barriers. Both types of barrier lead to S‐kinks in the J‐V curve and can be distinguished from each other and from other reasons for S‐kinks (e.g. imbalanced mobilities) by applying the presented approach. Furthermore, it is also helpful to investigate the driving forces for charge extraction in devices without S‐shaped J‐V curve close to open circuit to evaluate whether their electrodes are optimized.
GAPO syndrome: Four new patients with congenital glaucoma and myelinated retinal nerve fiber layer
Authors: Bozkurt Banu , Yıldırım Mahmut Selman , Okka Mehmet , Bitirgen Gülfidan
Published: March 2013

This article reports on the ophthalmological features of four Turkish children with GAPO syndrome, a very rare autosomal recessive condition characterized by growth retardation (G), alopecia (A), pseudoanodontia (P) (failure of tooth eruption), and optic atrophy (O). The children were from two unrelated families born to consanguineous parents. They had the characteristic facial appearance of alopecia, rarefaction of eyebrows and eyelashes, frontal bossing, high forehead, midfacial hypoplasia, hypertelorism, and thickened eyelids and lips. Two children had severe end‐stage glaucoma in both eyes and unilateral corneal opacity, whereas other two children had myelinated retinal nerve fiber layer; one with bilateral optic atrophy and the other one with persistent pupillary membrane in the left eye. © 2013 Wiley Periodicals, Inc.
Partial deletion of ANKRD11 results in the KBG phenotype distinct from the 16q24.3 microdeletion syndrome
Authors: Khalifa Mohamed , Stein Jennifer , Grau Lance , Nelson Valery , Meck Jeanne , Aradhya Swaroop , Duby John
Published: March 2013

KBG syndrome (OMIM 148050) is a very rare genetic disorder characterized by macrodontia, distinctive craniofacial abnormalities, short stature, intellectual disability, skeletal, and neurologic involvement. Approximately 60 patients have been reported since it was first described in 1975. Recently mutations in ANKRD11 have been documented in patients with KBG syndrome, and it has been proposed that haploinsufficiency of ANKRD11 is the cause of this syndrome. In addition, copy number variation in the 16q24.3 region that includes ANKRD11 results in a variable phenotype that overlaps with KBG syndrome and also includes autism spectrum disorders and other dysmorphic facial features. In this report we present a 2½‐year‐old African American male with features highly suggestive of KBG syndrome. Genomic microarray identified an intragenic 154 kb deletion at 16q24.3 within ANKRD11. This child's mother was mosaic for the same deletion (present in approximately 38% of cells) and exhibited a milder phenotype including macrodontia, short stature and brachydactyly. This family provides additional evidence that ANKRD11 causes KBG syndrome, and the mild phenotype in the mosaic form suggests that KBG phenotypes might be dose dependent, differentiating it from the more variable 16q24.3 microdeletion syndrome. This family has additional features that might expand the phenotype of KBG syndrome. © 2013 Wiley Periodicals, Inc.
Epilepsy in children with trisomy 18
Authors: Kumada Tomohiro , Maihara Toshiro , Higuchi Yoshihisa , Nishida Yoshinobu , Taniguchi Yoshihiro , Fujii Tatsuya
Published: March 2013

Although the reported incidence of epilepsy associated with trisomy 18 is 25–50%, there have been no detailed descriptions of the characteristics of trisomy 18‐related epilepsy. We investigated the characteristics of epilepsy in children with trisomy 18 who remained alive for over 1 year by sending questionnaires to pediatric neurologists belonging to the Kyoto Multi‐institutional Study Group of Pediatric Neurology. Eleven patients with trisomy 18 were enrolled (age at the study, from 15 to 134 months; median, 43 months), of whom seven (64%) had epilepsy. The age at seizure onset ranged from 1 to 42 months (median: 11 months). Among the seven patients with epilepsy, two had focal epilepsy, four had generalized epilepsy including infantile spasms in three, and the remaining one had an unclassified type. Seizure seminology included complex partial seizures in both the patients with focal epilepsy. At the time of the investigation, three children with generalized epilepsy still had daily seizures, while the remaining four were seizure‐free. In conclusion, the characteristics of epilepsy in patients with trisomy 18 were as follows: over half of the children developed epilepsy during infancy or early childhood; infantile spasms might be one of the common epileptic syndromes; the epilepsy was intractable in half of the children, especially in those with generalized epilepsy. © 2013 Wiley Periodicals, Inc.
Artery tortuosity syndrome exhibiting early‐onset emphysema with novel compound heterozygous SLC2A10 mutations
Authors: Takahashi Yoshiko , Fujii Katsunori , Yoshida Akiko , Morisaki Hiroko , Kohno Yoichi , Morisaki Takayuki
Published: March 2013

We report on a 2‐year‐old Japanese boy with early‐onset pulmonary emphysema, exhibiting dysmorphic face, loose skin, and inguinal and Morgagni hernias. He was admitted to our hospital owing to refractory respiratory infection. On the basis of his clinical features, we investigated the SLC2A10 gene and identified novel compound heterozygous mutations of c.417T > A and c.692G > A, leading to the diagnosis of artery tortuosity syndrome (ATS). This syndrome is an extremely rare autosomal recessive disorder characterized by tortuosity and elongation of the large and medium‐sized arteries, hyperextensible skin, and diverse hernias, mostly reported from Europe and Middle Eastern countries, but not from Asia. Although chronic obstructive pulmonary disease, namely, emphysema, has not been well documented in ATS, it may be likely because TGF‐beta up‐regulation is known to be evoked by SLC2A10 mutations, resulting in reconstruction of pulmonary endothelial cells and emphysema. This is the first report of ATS associated with early‐onset pulmonary emphysema, suggesting that patients with ATS may also require close attention for chronic obstructive pulmonary disease. © 2013 Wiley Periodicals, Inc.
Narrowing the Critical Region for Congenital Vertical Talus in Patients With Interstitial 18q Deletions
Authors: Mark Paul R. , Radlinski Brian C. , Core Nathalie , Fryer Alan , Kirk Edwin P. , Haldeman‐Englert Chad R.
Published: March 2013

Interstitial deletions of 18q lead to a number of phenotypic features, including multiple types of foot deformities. Many of these associated phenotypes have had their critical regions narrowly defined. Here we report on three patients with small overlapping deletions of chromosome 18q determined by microarray analysis (chr18:72493281–73512553 hg19 coordinates). All of the patients have congenital vertical talus (CVT). Based on these findings and previous reports in the literature and databases, we narrow the critical region for CVT to a minimum of five genes (ZNF407, ZADH2, TSHZ1, C18orf62, and ZNF516), and propose that TSHZ1 is the likely causative gene for CVT in 18q deletion syndrome. © 2013 Wiley Periodicals, Inc.
Keratoconus in Costello Syndrome
Authors: Gripp Karen W. , Demmer Laurie A.
Published: March 2013

Keratoconus is a corneal dystrophy with progressive corneal thinning resulting in abnormal corneal shape and astigmatism. Corneal hydrops and rupture can occur and corneal transplant may become necessary. While keratoconus is rare in the general population occurring in about 1/2,000 individuals, it is more common in individuals with intellectual disability and syndromic conditions. Connective tissue abnormalities, most typically brittle cornea syndrome, have frequently been reported in association with keratoconus. Here, we report on bilateral keratoconus with acute hydrops in the left eye of a 24‐year‐old male with Costello syndrome. The patient was treated medically. After resolution of the hydrops, he had significant visual impairment from the resulting irregular astigmatism and scarring. This is the second report of keratoconus in Costello syndrome, suggesting an increased risk for this corneal dystrophy in individuals with Costello syndrome. Ongoing ophthalmological surveillance may be necessary for adult individuals with Costello syndrome, and apparent vision changes should be evaluated expediently. © 2013 Wiley Periodicals, Inc.
Evaluation of Chromosome 11p Imbalances in Aniridia and Wilms Tumor Patients
Authors: Busch Maike , Leube Barbara , Thiel Anne , Schanze Ina , Beier Manfred , Royer‐Pokora Brigitte
Published: March 2013

Newborn sporadic aniridia patients with an 11p13 deletion including the WT1 gene have an increased risk to develop Wilms tumor. At present a risk for Wilms tumor cannot be estimated in patients with deletions not extending into, but ending close to WT1. Therefore, it is important to determine the distance of deletion endpoints from the WT1 gene and survey these patients for a longer follow‐up time to obtain a more defined risk estimation. Using molecular methods, such as Multiplex Ligation‐dependent Probe Amplification (MLPA), deletion endpoints can be mapped more accurately than with FISH. We describe here the analysis of six aniridia patients, in two of these the deletions extend close to the 3′ end of WT1. At the ages of 3.8 and 4 years they have not developed a Wilms tumor, suggesting a low tumor risk in such patients. In addition we have studied 24 non‐AN cases with a higher likelihood for WT1 alterations with MLPA and found no deletions. In conclusion newborns with aniridia should be studied with molecular methods that can determine deletion endpoints in 11p13 exactly. For a better Wilms tumor risk estimation cases with deletion endpoints close to WT1 should be followed for at least 4–5 years. Furthermore germ line intragenic deletions affecting WT1 in patients with a higher likelihood for a WT1 association, for example, bilateral tumors, genitourinary aberrations, or nephrotic syndrome, were not found in this study, suggesting that deletions are rare events. © 2013 Wiley Periodicals, Inc.
Reduces Bone Mass as in Human Apert Syndrome
Authors: Zhou Xia , Pu Dongquan , Liu Ri , Li Xiangjie , Wen Xiujie , Zhang Li , Chen Lin , Deng Manjing , Liu Luchuan
Published: March 2013

Apert syndrome is a common craniosynostosis caused by gain‐of‐function missense mutations of fibroblast growth factor receptor 2 (FGFR2). Mice with the FGFR2 S252W mutation can elucidate the mechanism by which the human Apert syndrome phenotypes arise. However, many studies have focused on mutant skull and long bone malformation, only few studies have focused on mandible changes. Bone formation and micro‐architecture between 28‐ and 56‐day‐old mutant mice and controls were compared to investigate the changes in the mandibular micro‐architecture caused by the Fgfr2S252W/+ mutation to provide a basis for exploring the pathogenesis and therapeutic measures of human Apert syndrome. Fgfr2S252W/+ mutant mice were established, and their general characteristics, including weight, naso‐anal length, and calcium and phosphate content in serum and bone were tested. Calcein labeling, tartrate‐resistant acid phosphatase staining and toluidine blue staining were used to detect osteoblast and osteoclast activities. H&E staining and micro‐CT detection were used to test micro‐architecture changes. The changes in mineral apposition rate and micro‐architecture of the Fgfr2S252W/+ mice were statistically significant; however, the magnitude of the micro‐architecture became less with age. The Fgfr2S252W/+ mutation may retard mandibular bone formation, decreased bone volume, and compromised skeletal architecture by regulating both osteoblastogenesis and osteoclastogenesis. © 2013 Wiley Periodicals, Inc.
Brain–machine interface in chronic stroke rehabilitation: A controlled study
Authors: Ramos‐Murguialday Ander , Broetz Doris , Rea Massimiliano , Läer Leonhard , Yilmaz Özge , Brasil Fabricio L. , Liberati Giulia , Curado Marco R. , Garcia‐Cossio Eliana , Vyziotis Alexandros , Cho Woosang , Agostini Manuel , Soares Ernesto , Soekadar Surjo , Caria Andrea , Cohen Leonardo G. , Birbaumer Niels
Published: March 2013

Objective

Chronic stroke patients with severe hand weakness respond poorly to rehabilitation efforts. Here, we evaluated efficacy of daily brain–machine interface (BMI) training to increase the hypothesized beneficial effects of physiotherapy alone in patients with severe paresis in a double‐blind sham‐controlled design proof of concept study.

Methods

Thirty‐two chronic stroke patients with severe hand weakness were randomly assigned to 2 matched groups and participated in 17.8 ± 1.4 days of training rewarding desynchronization of ipsilesional oscillatory sensorimotor rhythms with contingent online movements of hand and arm orthoses (experimental group, n = 16). In the control group (sham group, n = 16), movements of the orthoses occurred randomly. Both groups received identical behavioral physiotherapy immediately following BMI training or the control intervention. Upper limb motor function scores, electromyography from arm and hand muscles, placebo–expectancy effects, and functional magnetic resonance imaging (fMRI) blood oxygenation level–dependent activity were assessed before and after intervention.

Results

A significant group × time interaction in upper limb (combined hand and modified arm) Fugl–Meyer assessment (cFMA) motor scores was found. cFMA scores improved more in the experimental than in the control group, presenting a significant improvement of cFMA scores (3.41 ± 0.563‐point difference, p = 0.018) reflecting a clinically meaningful change from no activity to some in paretic muscles. cFMA improvements in the experimental group correlated with changes in fMRI laterality index and with paretic hand electromyography activity. Placebo–expectancy scores were comparable for both groups.

Interpretation

The addition of BMI training to behaviorally oriented physiotherapy can be used to induce functional improvements in motor function in chronic stroke patients without residual finger movements and may open a new door in stroke neurorehabilitation. ANN NEUROL 2013;74:100–108
Circulating MicroRNAs as biomarkers for disease staging in multiple sclerosis
Authors: Gandhi Roopali , Healy Brian , Gholipour Taha , Egorova Svetlana , Musallam Alexander , Hussain Mohammad Shuja , Nejad Parham , Patel Bonny , Hei Hillary , Khoury Samia , Quintana Francisco , Kivisakk Pia , Chitnis Tanuja , Weiner Howard L.
Published: March 2013

Objective

MicroRNAs (miRNAs) are single‐stranded, small noncoding RNAs that regulate gene expression. Because they are stable in serum, they are being developed as biomarkers for cancer and other diseases. In multiple sclerosis (MS), miRNAs have been studied in cell populations but not in the circulation. In MS, a major challenge is to develop immune biomarkers to monitor disease. We asked whether circulating miRNAs could be identified in MS and whether they are linked to disease stage and/or disability.

Methods

A total of 368 miRNAs were measured in ethylenediaminetetraacetic acid plasma in 10 relapsing–remitting MS (RRMS) patients, 9 secondary progressive MS (SPMS) patients, and 9 healthy controls (HCs) using miRCURY LNA™ Universal RT microRNA polymerase chain reaction panels. Nineteen miRNAs from this discovery set were validated using qPCR on an independent set of 50 RRMS patients, 51 SPMS patients, and 32 HCs.

Results

We found that circulating miRNAs are differentially expressed in RRMS and SPMS versus HCs and in RRMS versus SPMS. We also found miRNAs to be linked to Expanded Disability Status Scale (EDSS). hsa‐miR‐92a‐1* was identified in the largest number of comparisons. It was different in RRMS versus SPMS, and RRMS versus HCs, and showed an association with EDSS and disease duration. miR‐92 has target genes involved in cell cycle regulation and cell signaling. The let‐7 family of miRNAs differentiated SPMS from HCs and RRMS from SPMS. let‐7 miRNAs regulate stem cell differentiation and T cell activation, activate Toll‐like receptor 7, and are linked to neurodegeneration. hsa‐miR‐454 differentiated RRMS from SPMS, and hsa‐miR‐145 differentiated RRMS from HCs and RRMS from SPMS. Interestingly, the same circulating miRNAs (let‐7 and miR‐92) that were differentially expressed in RRMS versus SPMS also differentiated amyotrophic lateral sclerosis (ALS) from RRMS subjects, but were not different between SPMS and ALS, suggesting that similar processes may occur in SPMS and ALS.

Interpretation

Our results establish circulating miRNAs as a readily accessible blood biomarker to monitor disease in MS. ANN NEUROL 2013;73:729–740
Organische Proteinchemie und ihre Anwendung für Markierungen und Engineering in Lebendzellsystemen
Authors: Takaoka Yousuke , Ojida Akio , Hamachi Itaru
Published: March 2013

Die Modifizierung von Proteinen mit synthetischen Sonden kann in vielfältiger Weise zur Aufklärung und Beeinflussung von Proteinfunktionen in vitro, in lebenden Zellen oder in vivo genutzt werden. Wir stellen hier die jüngsten Fortschritte bei den Methoden zur chemischen Proteinmodifizierung und ihrer Anwendung beim Protein‐Engineering vor, wobei wir uns auf vier Strategien konzentrieren: 1) Biokonjugationsreaktionen von Aminosäuren an der Oberfläche natürlicher Proteine, hauptsächlich im Reagenzglas; 2) bioorthogonale Reaktionen von Proteinen mit nichtnatürlichen reaktiven Markergruppen; 3) Kupplung von Erkennungs‐ und reaktiven Regionen unter Verwendung eines Paars aus Enzym oder kurzem Peptid mit einem Sondenmarker zur Markierung natürlicher Aminosäuren 4) ligandendirigierte chemische Reaktionen zur selektiven Markierung endogener Proteine in lebenden Systemen. Zusammengenommen bieten diese Techniken ein nützliches Repertoire von Methoden zur Anwendung in der chemischen Biologie, wobei die drei letztgenannten auf unveränderte (lebende) Systeme anwendbar sind. Die organische Chemie zur Manipulation endogener Proteine ist zwar noch in einer frühen Phase, doch die nachfolgend beschriebenen Anwendungen sind ein lohnendes Ziel für Chemiker.
Repeat hepatectomy for recurrent colorectal metastases
Authors: Wicherts D A , de Haas R J , Salloum C , Andreani P , Pascal G , Sotirov D , Adam R , Castaing D , Azoulay D
Published: March 2013

Background

The oncological benefit of repeat hepatectomy for patients with recurrent colorectal metastases is not yet proven. This study assessed the value of repeat hepatectomy for these patients within current multidisciplinary treatment.

Methods

Consecutive patients treated by repeat hepatectomy for colorectal metastases between January 1990 and January 2010 were included. Patients undergoing two-stage hepatectomy were excluded. Postoperative outcome was analysed and compared with that of patients who had only a single hepatectomy.

Results

A total of 1036 patients underwent 1454 hepatectomies for colorectal metastases. Of these, 288 patients had 362 repeat hepatectomies for recurrent metastases. Some 225 patients (78·1 per cent) had two hepatectomies, 52 (18·1 per cent) had three hepatectomies, and 11 patients (3·8 per cent) had a fourth hepatectomy. Postoperative morbidity following repeat hepatectomy was similar to that after initial liver resection (27·1 per cent after first, 34·4 per cent after second and 33·3 per cent after third hepatectomy) (P = 0·069). The postoperative mortality rate was 3·1 per cent after repeat hepatectomy versus 1·6 per cent after first hepatectomy. Three- and 5-year overall survival rates following first hepatectomy in patients who underwent repeat hepatectomy were 76 and 54 per cent respectively, compared with 58 and 45 per cent in patients who had only one hepatectomy (P = 0·003). In multivariable analysis, repeat hepatectomy performed between 2000 and 2010 was the sole independent factor associated with longer overall survival.

Conclusion

Repeat hepatectomy for recurrent colorectal metastases offers long-term survival in selected patients.
Towards Squalamine Mimics: Synthesis and Antibacterial Activities of Head‐to‐Tail Dimeric SterolPolyamine Conjugates
Authors: Chen Wen‐Hua , Wennersten Christine , Moellering Robert C. , Regen Steven L.
Published: March 2013

Four dimeric sterolpolyamine conjugates have been synthesized from the homo‐ and hetero‐connection of monomeric sterolpolyamine analogs in a head‐to‐tail manner. These dimeric conjugates show strong antibacterial activity against a broad spectrum of Gram‐positive bacteria, whereas their corresponding activities against Gram‐negative bacteria are relatively moderate. Though no significant difference was observed in the activities of these conjugates, cholic acid‐containing dimeric conjugates generally exhibit higher activities than the corresponding deoxycholic acid‐derived analogs. This is in contrast to the finding that a monomeric deoxycholic acid‐spermine conjugate was more active than the corresponding cholic acid‐derived analog.
Chemical Diversity of the Natural Populations of Dalmatian Pyrethrum (Tanacetum cinerariifolium (Trevir.) Sch.Bip.) in Croatia
Authors: Grdiša Martina , Babić Sandra , Periša Martina , Carović‐Stanko Klaudija , Kolak Ivan , Liber Zlatko , Jug‐Dujaković Marija , Satovic Zlatko
Published: March 2013

Dalmatian pyrethrum (Tanacetum cinerariifolium (Trevir.) Sch.Bip.) is a plant species endemic to the east Adriatic coast. The bioactive substance of Dalmatian pyrethrum is a natural insecticide, pyrethrin, a mixture of six active components (pyrethrins I and II, cinerins I and II, and jasmolins I and II). The insecticidal potential of pyrethrin was recognized decades ago, and dried and ground flowers have traditionally been used in Croatian agriculture and households.

A total of 25 Dalmatian pyrethrum populations from Croatia were studied to determine the pyrethrin content and composition, and to identify distinct chemotypes. The total pyrethrin content ranged from 0.36 to 1.30% (dry flower weight; DW) and the pyrethrin I/pyrethrin II ratio ranged from 0.64 to 3.33%. The statistical analyses revealed that the correlations between the percentage of pyrethrin I and of all the other components were significant and negative. The total pyrethrin content was positively correlated with the percentage of pyrethrin I and negatively correlated with cinerin II. The multivariate analysis of the chemical variability enabled the identification of five chemotypes among 25 Dalmatian pyrethrum populations. The chemical characterization of indigenous Dalmatian pyrethrum populations may serve as a good background for future breeding and agricultural exploitation.
Essential‐Oil Composition of Helichrysum italicum (Roth) G.Don ssp. italicum from Elba Island (Tuscany, Italy)
Authors: Leonardi Michele , Ambryszewska Katarzyna E. , Melai Bernardo , Flamini Guido , Cioni Pier Luigi , Parri Federico , Pistelli Luisa
Published: March 2013

The composition of 21 essential‐oil samples isolated from Helichrysum italicum collected in seven locations of Elba Island (Tuscany, Italy), characterized by different soil types, during three different periods (January, May, and October 2010) was determined by GC‐FID and GC/EI‐MS analyses. In total, 115 components were identified, representing 96.8–99.8% of the oil composition. The oils were characterized by a high content of oxygenated monoterpenes (38.6–62.7%), while monoterpene and sesquiterpene hydrocarbons accounted for 2.3–41.9 and 5.1–20.1% of the identified constituents, respectively. The main oxygenated derivatives were nerol (2.8–12.8%) and its ester derivative neryl acetate (5.6–45.9%). To compare the chemical variability of the species within Elba Island and between the island and other localities within the Mediterranean area, studied previously, multivariate statistical analysis was performed. The results obtained showed a difference in the composition of the essential oils of H. italicum from Elba Island, mainly due to the environment where the plant grows, and, in particular, to the soil type. These hypotheses were further confirmed by the comparison of these oils with essential oils obtained from H. italicum collected on other islands of the Tuscan archipelago.
Rh1/γ‐Al2O3 Single‐Atom Catalysis of O2 Activation and CO Oxidation: Mechanism, Effects of Hydration, Oxidation State, and Cluster Size
Authors: Ghosh Tushar K. , Nair Nisanth N.
Published: March 2013

The single‐atom catalysis of O2 activation and CO oxidation with Rh1 supported on γ‐Al2O3 is investigated here through ab initio molecular dynamics techniques. We scrutinize the molecular details of the mechanism for the full catalytic cycle that involves the oxidation of two CO molecules in succession. The effect of the surface hydration and oxidation state of Rh on the kinetics of O2 activation and CO oxidation is presented. We also report here the catalytic activity of experimentally intercepted RhI(CO)2 on γ‐Al2O3. Furthermore, we delineate the importance of single‐atom catalysis by comparing the performance of the Rh6/Al2O3 catalyst. A molecular level understanding of the differential reactivity on hydration, on oxidation, and of a larger Rh cluster size is reported.
Painting Anatase (TiO2) Nanocrystals on Long Nanofibers to Prepare Photocatalysts with Large Active Surface for Dye Degradation and Organic Synthesis
Authors: Zheng Zhanfeng , Zhao Jian , Liu Hongwei , Liu Jiangwen , Bo Arixin , Zhu Huaiyong
Published: March 2013

Anatase TiO2 nanocrystals were painted on H‐titanate nanofibers by using an aqueous solution of titanyl sulfate. The anatase nanocrystals were bonded solidly onto the titanate fibers through formation of coherent interfaces at which the oxygen atoms were shared by the nanocrystals and the fiber. This approach allowed us to create large anatase surfaces on the nanofibers, which are active in photocatalytic reactions. This method was also applied successfully to coat anatase nanocrystals on surfaces of fly ash and layered clay. The painted nanofibers exhibited a much higher catalytic activity for the photocatalytic degradation of sulforhodamine B and the selective oxidation of benzylamine to the corresponding imine (with a product selectivity >99 %) under UV irradiation than both the parent H‐titanate nanofibers and a commercial TiO2 powder, P25. We found that gold nanoparticles supported on H‐titanate nanofibers showed no catalytic activity for the reduction of nitrobenzene to azoxybenzene, whereas the gold nanoparticles supported on the painted nanofibers and P25 could efficiently reduce nitrobenzene to azoxybenzene as the sole product under visible light irradiation. These results were different from those from the reduction on the gold nanoparticles photocatalyst on ZrO2, in which the azoxybenzene was the intermediate and converted to azobenzene quickly. Evidently, the support materials significantly affect the product selectivity of the nitrobenzene reduction. Finally, the new photocatalysts could be easily dispersed into and separated from a liquid because of their fibril morphology, which is an important advantage for practical applications.
Synthesis and Redox and Photophysical Properties of Benzodifuran–Spiropyran Ensembles
Authors: Li Hui , Ding Jie , Chen Songjie , Beyer Christoph , Liu Shi‐Xia , Wagenknecht Hans‐Achim , Hauser Andreas , Decurtins Silvio
Published: March 2013

Two benzodifuran (BDF)‐coupled spiropyran (SP) systems and their BDF reference compounds were obtained in good yields through Huisgen–Meldal–Sharpless “click” chemistry and then subjected to investigation of their electrochemical and photophysical properties. In both SP and merocyanine (MC) forms of the coupled molecules, the BDF‐based emission is quenched to around 1 % of the quantum yield of emission from the BDF reference compounds. Based on electrochemical data, this quenching is attributed to oxidative electron‐transfer quenching. Irradiation at 366 nm results in ring opening to the MC forms of the BDF‐coupled SP compounds and the SP reference compound with a quantum efficiency of about 50 %. The rate constants for the thermal ring closing are approximately 3.4×10−3 s−1. However, in the photostationary states the MC fractions of the coupled molecules are substantially lower than that of the reference SP compound, attributed to the observed acceleration of the ring‐closing reaction upon irradiation. As irradiation at 366 nm invariably also excites higher‐energy transitions of the BDF units in the coupled compounds, the ring‐opening reaction is accelerated relative to the SP reference, which results in lower MC fractions in the photostationary state. Reversible photochromism of these BDF‐coupled SP compounds renders them promising in the field of molecular switches.