bluuebunny/crossref_metadata_2025_split huggingface dataset:
A family with a complex clinical presentation characterized by arrhythmogenic right ventricular dysplasia/cardiomyopathy and features of branchio‐oculo‐facial syndrome
Authors: Murray Brittney , Wagle Rohan , Amat‐Alarcon Nuria , Wilkens Alisha , Stephens Paul , Zackai Elaine H. , Goldmuntz Elizabeth , Calkins Hugh , Deardorff Matthew A. , Judge Daniel P. 
Published: January 2013

AbstractArrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a familial form of cardiomyopathy typically caused by mutations in genes that encode an element of the cardiac desmosome. Branchio‐oculo‐facial syndrome (BOFS) is a craniofacial disorder caused by TFAP2A mutations. In a family segregating ARVD/C, some members also had features of BOFS. Genetic testing for ARVD/C identified a mutation in PKP2, encoding plakophilin‐2, a component of the cardiac desmosome. Evaluation of dysmorphology by chromosome microarray (CMA) identified a 4.4 Mb deletion at chromosome 6p24 that included both TFAP2A and DSP, encoding desmoplakin, an additional component of the cardiac desmosome implicated in ARVD/C. A family member with both the 6p24 deletion and PKP2 mutation had more severe cardiac dysfunction. These findings suggest that this contiguous gene deletion contributes to both ARVD/C and BOFS, and that DSP haploinsufficiency may contribute to cardiomyopathy. This family provides a clinical example that underscores the need for careful evaluation in clinical scenarios where genetic heterogeneity is known to exist. Finally, it suggests that individuals with unexplained cardiomyopathy and dysmorphic facial features may benefit from CMA analysis. © 2013 Wiley Periodicals, Inc.
The association between allergic rhinitis and sleep‐disordered breathing in children: a systematic review
Authors: Lin Sandra Y. , Melvin Thuy‐Anh N. , Boss Emily F. , Ishman Stacey L. 
Published: January 2013

BackgroundThe objective of this work was to systematically review existing literature on the association between allergic rhinitis (AR) and sleep‐disordered breathing (SDB) in children.MethodsWe performed a literature search encompassing the last 25 years in PubMed, EMBASE, and Cochrane CENTRAL. Inclusion criteria included English‐language papers containing original human data, number of subjects ≥7, and age <18 years old. Data was systematically collected on study design, patient demographics, clinical characteristics/outcomes, and level‐of‐evidence. Two investigators independently reviewed all articles.ResultsThe initial search yielded 433 abstracts, of which 18 articles were included. Twelve (67%) of the 18 articles showed a statistically significant association between AR and SDB. All articles were either case‐series or case‐control studies. Based on the Newcastle‐Ottawa scale, the quality of the articles was determined to be fair to good. For characterizing AR, 7 (39%) studies included skin‐prick testing and/or in vitro testing. For determining presence of SDB, 7 (39%) of the studies used polysomnographic data, of which 1 study incorporated data from a home polysomnogram. Habitual snoring was the most common form of SDB studied, in 10 (56%) of the articles. Obstructive sleep apnea was studied in 6 (33%) articles.ConclusionAlthough the majority of the studies included in this review showed a significant association between AR and SDB, all of the studies were evidence level 3b and 4, for an overall grade of B− evidence (Oxford Evidence‐Based Medicine Center). Further higher‐quality studies should be performed in the future to better evaluate the relationship between AR and SDB in children.
Predicting population status of freshwater pearl mussel (Margaritifera margaritifera, L.) in central Sweden using instream and riparian zone land‐use data
Authors: Degerman E. , Andersson K. , Söderberg H. , Norrgrann O. , Henrikson L. , Angelstam P. , Törnblom J. 
Published: January 2013

ABSTRACT

The freshwater pearl mussel Margaritifera margaritifera (L.) has become scarce and threatened throughout most of its distribution range, and is consequently listed as ‘endangered’ by the IUCN. It has been suggested and used as an indicator species and even as an umbrella species. Despite this, few studies have examined factors affecting its catchment‐level population status.
This work tested the hypothesis that it is possible to predict freshwater pearl mussel population viability (reproducing/not reproducing) and population status in central Sweden from a combination of geospatial data describing riparian land‐cover and use, water chemistry data, and electrofishing data describing the abundance of the host fish species (brown trout, Salmo trutta) in 56 streams.
Mussel population viability was best predicted by total phosphorus. Using higher‐resolution data (six classes) on mussel population status, host fish abundance (0+ trout) was the best predictor. At abundances below 5 0+ trout per 100 m2 and above a concentration of 8 µg L–1 total phosphorus, only three viable populations existed.
Geospatial data could be used to predict levels of total phosphorus and thereby indirectly to predict mussel viability. Geospatial data could not be used to predict abundance of 0+ trout, which is thought to depend more on instream factors. This study demonstrates that geospatial data on riparian land‐use and cover can be a useful tool for screening waters potentially harbouring non‐reproducing mussel populations. Copyright © 2013 John Wiley & Sons, Ltd.
Nuclear Accumulation of Prohibitin 1 in Osteoarthritic Chondrocytes Down‐Regulates PITX1 Expression
Authors: Picard Cynthia , Pellicelli Martin , Taheri Maryam , Lavoie Jean‐Francois , Doucet Roxanne , Wang DaShen , Bernard Lauriane , Bouhanik Saadallah , Lavigne Patrick , Moreau Alain 
Published: January 2013

AbstractObjectiveTo decipher the molecular mechanisms down‐regulating PITX1 expression in primary osteoarthritis (OA).MethodsThe functional activity of different PITX1 promoter regions was assessed by luciferase reporter assay. Tandem mass spectrometry coupled to protein sequencing was performed using nuclear extracts prepared from OA chondrocytes, in order to identify proteins bound to DNA regulatory elements. Expression analyses of selected candidate proteins were performed by real‐time reverse transcription–polymerase chain reaction (RT‐PCR) and immunohistochemistry methods, using cartilage sections and articular chondrocytes from non‐OA control subjects and patients with OA. Gain‐of‐function and loss‐of‐function experiments were performed in normal and OA chondrocytes, respectively, to study their effects on PITX1 regulation. The results were validated by real‐time RT‐PCR and immunohistochemistry in STR/Ort mice, a well‐known animal model of OA.ResultsPITX1 promoter analyses led to the identification of prohibitin 1 (PHB1) bound to a distal E2F1 transcription factor site. Aberrant accumulation of PHB1 was detected in the nuclei of OA articular chondrocytes, and overexpression of PHB1 in control cells was sufficient to inhibit endogenous PITX1 expression at the messenger RNA and protein levels. Conversely, knockdown of PHB1 in OA articular chondrocytes resulted in up‐regulation of PITX1. Studies of early molecular changes in STR/Ort mice revealed a similar nuclear accumulation of PHB1, which correlated with Pitx1 repression.ConclusionCollectively, these data define an unrecognized role for PHB1 in repressing PITX1 expression in OA chondrocytes.
Coding Scheme Based on Boundary Functions for Distributed System with Finite Channel Data Rates and Packet Dropouts
Authors: Jian Wang , Zhibin Yan , Jiafeng Yu 
Published: January 2013

AbstractThis paper considers discrete linear systems with multiple distributed sensors that are connected to one controller via channels of finite data rate. Each encoder has access to only one component of the current system output vector. By the proposed boundary function method, a condition expressed in terms of output feedback gain matrix, the parameters of boundary functions, and the channel data rates is given to guarantee the convergence of the state in the case of reliable channels. Then, we extend the result to the case of erasure channels.
Self‐Repairing Control Against Unknown Sensor Failures and Biases
Authors: Takahashi Masanori 
Published: January 2013

AbstractIn this paper, a self‐repairing control system (SRCS) is presented for a plant with unknown sensor failure and bias. The simultaneous use of an unstable filter and a differential filter makes a monitored signal hit the prescribed threshold whenever the failure of a stuck‐type occurs. Then, the failed sensor can be found exactly, and be switched to the healthy backup. The advantage of the method is that any information about the plants for fault detection is not necessary. Also, the introduction of the differential filter can eliminate the effect of the sensor bias. Thus, the SRCS copes with the problems on both sensor failures and biases. Through several numerical simulations, the effectiveness of the proposed SRCS is confirmed.
Consensus analysis of continuous‐time second‐order multi‐agent systems with nonuniform time‐delays and switching topologies
Authors: Zhang Wenguang , Liu Jizhen , Zeng Deliang , Yang Tingting 
Published: January 2013

AbstractIn this study, consensus problems for second‐order multi‐agent systems with nonuniform and switching topologies are investigated. Each agent has a self‐delay, and each delay is independent of the others. As a measure of the disagreement dynamics, a class of positive semi‐definite Lyapunov–Krasovskii functions are introduced. Using algebraic graph theory and these Lyapunov–Krasovskii functions, sufficient conditions are derived by contradiction under which all agents asymptotically reach consensus. Finally, the effectiveness of the obtained theoretical results is demonstrated through numerical simulations.
Curcumin and liver disease
Authors: Vera‐Ramirez Laura , Pérez‐Lopez Patricia , Varela‐Lopez Alfonso , Ramirez‐Tortosa MCarmen , Battino Maurizio , Quiles José L. 
Published: January 2013

AbstractLiver diseases pose a major medical problem worldwide and a wide variety of herbs have been studied for the management of liver‐related diseases. In this respect, curcumin has long been used in traditional medicine, and in recent years it has been the object of increasing research interest. In combating liver diseases, it seems clear that curcumin exerts a hypolipidic effect, which prevents the fatty acid accumulation in the hepatocytes that may result from metabolic imbalances, and which may cause nonalcoholic steatohepatitis. Another crucial protective activity of curcumin, not only in the context of chronic liver diseases but also regarding carcinogenesis and other age‐related processes, is its potent antioxidant activity, which affects multiple processes and signaling pathways. The effects of curcumin on NF‐κβ are crucial to our understanding of the potent hepatoprotective role of this herb‐derived micronutrient. Because curcumin is a micronutrient that is closely related to cellular redox balance, its properties and activity give rise to a series of molecular reactions that in every case and biological situation affect the mitochondria. © 2013 BioFactors, 39(1):88–100, 2013
Curcumin in various cancers
Authors: Shehzad Adeeb , Lee Jaetae , Lee Young Sup 
Published: January 2013

AbstractCurcumin (diferuloylmethane), an active constituent of turmeric, is a well‐described phytochemical, which has been used since ancient times for the treatment of various diseases. The dysregulation of cell signaling pathways by the gradual alteration of regulatory proteins is the root cause of cancers. Curcumin modulates regulatory proteins through various molecular mechanisms. Several research studies have provided in‐depth analysis of multiple targets through which curcumin induces protective effects against cancers including gastrointestinal, genitourinary, gynecological, hematological, pulmonary, thymic, brain, breast, and bone. The molecular mechanisms of action of curcumin in treating different types of cancers remain under investigation. The multifaceted role of this dietary agent is mediated through its inhibition of several cell signaling pathways at multiple levels. Curcumin has the ability to inhibit carcinogenicity through the modulation of the cell cycle by binding directly and indirectly to molecular targets including transcription factors (NF‐kB, STAT3, β‐catenin, and AP‐1), growth factors (EGF, PDGF, and VEGF), enzymes (COX‐2, iNOS, and MMPs), kinases (cyclin D1, CDKs, Akt, PKC, and AMPK), inflammatory cytokines (TNF, MCP, IL‐1, and IL‐6), upregulation of proapoptotic (Bax, Bad, and Bak) and downregulation of antiapoptotic proteins (Bcl2 and Bcl‐xL). A variety of animal models and human studies have proven that curcumin is safe and well tolerated even at very high doses. This study elaborates the current understanding of the chemopreventive effects of curcumin through its multiple molecular pathways and highlights its therapeutic value in the treatment and prevention of a wide range of cancers. © 2013 BioFactors, 39(1):56–68, 2013
Facile Synthesis of Thermal‐ and Photostable Titania with Paramagnetic Oxygen Vacancies for Visible‐Light Photocatalysis
Authors: Zou Xiaoxin , Liu Jikai , Su Juan , Zuo Fan , Chen Jiesheng , Feng Pingyun 
Published: January 2013

AbstractA novel dopant‐free TiO2 photocatalyst (Vo.‐TiO2), which is self‐modified by a large number of paramagnetic (single‐electron‐trapped) oxygen vacancies, was prepared by calcining a mixture of a porous amorphous TiO2 precursor, imidazole, and hydrochloric acid at elevated temperature (450 °C) in air. Control experiments demonstrate that the porous TiO2 precursor, imidazole, and hydrochloric acid are all necessary for the formation of Vo.‐TiO2. Although the synthesis of Vo.‐TiO2 originates from such a multicomponent system, this synthetic approach is facile, controllable, and reproducible. X‐ray diffraction, XPS, and EPR spectroscopy reveal that the Vo.‐TiO2 material with a high crystallinity embodies a mass of paramagnetic oxygen vacancies, and is free of other dopant species such as nitrogen and carbon. UV/Vis diffuse‐reflectance spectroscopy and photoelectrochemical measurement demonstrate that Vo.‐TiO2 is a stable visible‐light‐responsive material with photogenerated charge separation efficiency higher than N‐TiO2 and P25 under visible‐light irradiation. The Vo.‐TiO2 material exhibits not only satisfactory thermal‐ and photostability, but also superior photocatalytic activity for H2 evolution (115 μmol h−1 g−1) from water with methanol as sacrificial reagent under visible light (λ>400 nm) irradiation. Furthermore, the effects of reaction temperature, ratio of starting materials (imidazole:TiO2 precursor) and calcination time on the photocatalytic activity and the microstructure of Vo.‐TiO2 were elucidated.
Magnesium Catalysis of Imine Hydroboration
Authors: Arrowsmith Merle , Hill Michael S. , Kociok‐Köhn Gabriele 
Published: January 2013

AbstractThe β‐diketiminato magnesium alkyl complex [LMgnBu] (L=CH{CMe(NDipp)}2, Dipp=diisopropylphenyl) is shown to be a highly effective precatalyst for the hydroboration of alkyl and aryl substituted aldimines and ketimines with pinacol borane (HBpin). Catalysis is proposed to occur through a sequence of MgN/BH metathesis and rate‐determining MgH/NC insertion steps, a proposal strongly supported by stoichiometric studies and kinetic analysis. The reactions are observed to proceed through the intermediacy of well‐defined magnesium amides, two examples of which have been isolated and structurally characterized. Mechanistic investigations suggest that the catalytic rate‐determining process occurs at an isolated magnesium center and requires the presence of two molecules of the imine substrate for effective turnover. This latter observation is rationalized as a requirement for the secondary substrate molecule to displace HBpin from the coordination sphere of the catalytic magnesium center.
Dynamic Electrosorption Analysis as an Effective Means to Characterise the Structure of Bulk Graphene Assemblies
Authors: Zhu Junwu , Cheng Chi , Yang Xiaowei , Wang Yufei , Qiu Ling , Li Dan 
Published: January 2013

AbstractRestacking of graphene sheets to a graphite‐like structure is a prevailing problem that is known to compromise the performance of individual graphene sheets in an assembled bulk form. To address this common problem efficiently and monitor the structure and quality of graphene products comprehensively, it is highly desirable to develop reliable metrology techniques for characterising graphene‐based materials on a bulk assembly level and in a quantitative manner. Here, by revisiting the physicochemical principle of electrosorption, we propose a simple electrochemical approach, namely dynamic electrosorption analysis (DEA), as an easily accessible and effective technique for evaluation of the self‐stacking behaviour of graphene. Taking multilayered chemically converted graphene films as a model, we demonstrate that the DEA technique can effectively reveal very subtle variation in accessible surface area and pore size of graphene assemblies in the liquid phase and thus can provide useful insights to the experimental design relating to restacking control. This work also reveals the huge effect some routine processing conditions, such as heat treatment and drying, can have on the structure and performance of graphene‐based bulk materials, providing useful guidance for future manufacturing of this class of materials.
Controlled Stacking and Unstacking of Peripheral Chlorophyll Units Drives the Spring‐Like Contraction and Expansion of a Semi‐Artificial Helical Polymer
Authors: Numata Munenori , Kinoshita Daiki , Hirose Naoya , Kozawa Tomohiro , Tamiaki Hitoshi , Kikkawa Yoshihiro , Kanesato Masatoshi 
Published: January 2013

AbstractDeveloping new strategies for controlling polymer conformations through precise molecular recognition can potentially generate a machine‐like motion that is dependent on molecular information—an important process for the preparation of new intelligent nanomaterials (e.g., polymer‐based nanomachines) in the field bordering between polymer chemistry and conventional supramolecular sciences. Herein, we propose a strategy to endow a helical polymer chain with dynamic spring‐like (contraction/expansion) motion through the one‐dimensional self‐assembly (aggregation/disaggregation) of peripheral amphiphilic molecules. In this developing system, we employed a semi‐artificial helical polysaccharide presenting peripheral amphiphilic chlorophyll units as a power device that undergoes contractive motion in aqueous media, driven by strong π–π interactions of its chlorophyll units or by cooperative molecular recognition of bipyridyl‐type ligands through pairs of chlorophyll units, thereby converting molecular information into the regulated motion of a spring. In addition, this system also undergoes expansive motion through coordination of pyridine. We anticipate that this strategy will be applicable (when combined with the established wrapping chemistry of the helical polysaccharide) to the development of, for example, drug carriers (e.g., nano‐syringes), actuators (stimuli‐responsive films), and directional transporters (nano‐railways), thereby extending the frontiers of supramolecular science.
Fueling Open‐Source Drug Discovery: 177 Small‐Molecule Leads against Tuberculosis
Authors: Ballell Lluís , Bates Robert H. , Young Rob J. , Alvarez‐Gomez Daniel , Alvarez‐Ruiz Emilio , Barroso Vanessa , Blanco Delia , Crespo Benigno , Escribano Jaime , González Rubén , Lozano Sonia , Huss Sophie , Santos‐Villarejo Angel , Martín‐Plaza José Julio , Mendoza Alfonso , Rebollo‐Lopez María José , Remuiñan‐Blanco Modesto , Lavandera José Luis , Pérez‐Herran Esther , Gamo‐Benito Francisco Javier , García‐Bustos José Francisco , Barros David , Castro Julia P. , Cammack Nicholas 
Published: January 2013

AbstractWith the aim of fuelling open‐source, translational, early‐stage drug discovery activities, the results of the recently completed antimycobacterial phenotypic screening campaign against Mycobacterium bovis BCG with hit confirmation in M. tuberculosis H37Rv were made publicly accessible. A set of 177 potent non‐cytotoxic H37Rv hits was identified and will be made available to maximize the potential impact of the compounds toward a chemical genetics/proteomics exercise, while at the same time providing a plethora of potential starting points for new synthetic lead‐generation activities. Two additional drug‐discovery‐relevant datasets are included: a) a drug‐like property analysis reflecting the latest lead‐like guidelines and b) an early lead‐generation package of the most promising hits within the clusters identified.
Body mass index at diagnosis and survival among colon cancer patients enrolled in clinical trials of adjuvant chemotherapy
Authors: Sinicrope Frank A. , Foster Nathan R. , Yothers Greg , Benson Al , Seitz Jean Francois , Labianca Roberto , Goldberg Richard M. , DeGramont Aimery , O'Connell Michael J. , Sargent Daniel J. ,   for the Adjuvant Colon Cancer Endpoints (ACCENT) Group
Published: January 2013

AbstractBACKGROUND:Although obesity is an established risk factor for developing colon cancer, its prognostic impact and relation to patient sex in colon cancer survivors remains unclear.METHODS:The authors examined the prognostic and predictive impact of the body mass index (BMI) in patients with stage II and III colon carcinoma (N = 25,291) within the Adjuvant Colon Cancer Endpoints (ACCENT) database. BMI was measured at enrollment in randomized trials of 5‐fluorouracil–based adjuvant chemotherapy. Association of BMI with the time to recurrence (TTR), disease‐free survival (DFS), and overall survival (OS) were determined using Cox regression models. Statistical tests were 2‐sided.RESULTS:During a median follow‐up of 7.8 years, obese and underweight patients had significantly poorer survival compared with overweight and normal‐weight patients. In a multivariable analysis, the adverse prognostic impact of BMI was observed among men but not among women (Pinteraction= .0129). Men with class 2 and 3 obesity (BMI ≥35.0 kg/m2) had a statistically significant reduction in DFS (hazard ratio [HR], 1.16; 95% confidence interval [CI], 1.01‐1.33;P= .0297) compared with normal‐weight patients. Underweight patients had a significantly shorter TTR and reduced DFS (HR, 1.18; 95% CI, 1.09‐1.28;P< .0001) that was more significant among men (HR, 1.31; 95% CI, 1.15‐1.50;P< .0001) than among women (HR, 1.11; 95% CI, 1.01‐1.23;P= .0362;Pinteraction= .0340). BMI was not predictive of a benefit from adjuvant treatment.CONCLUSIONS:Obesity and underweight status were associated independently with inferior outcomes in patients with colon cancer who received treatment in adjuvant chemotherapy trials. Cancer 2013. © 2013 American Cancer Society.