bluuebunny/crossref_metadata_2025_split huggingface dataset:
Loss of junctophilin‐3 contributes to huntington disease‐like 2 pathogenesis
Authors: Seixas Ana I. , Holmes Susan E. , Takeshima Hiroshi , Pavlovich Amira , Sachs Nancy , Pruitt Jennifer L. , Silveira Isabel , Ross Christopher A. , Margolis Russell L. , Rudnicki Dobrila D. 
Published: August 2011

AbstractObjective:Huntington disease‐like 2 (HDL2) is a progressive, late onset autosomal dominant neurodegenerative disorder, with remarkable similarities to Huntington disease (HD). HDL2 is caused by a CTG/CAG repeat expansion. In the CTG orientation, the repeat is located within the alternatively spliced exon 2A of junctophilin‐3 (JPH3), potentially encoding polyleucine and polyalanine, whereas on the strand antisense to JPH3, the repeat is in frame to encode polyglutamine. The JPH3 protein product serves to stabilize junctional membrane complexes and regulate neuronal calcium flux. We have previously demonstrated the potential pathogenic properties of JPH3 transcripts containing expanded CUG repeats. The aim of this study was to test the possibility that loss of JPH3 expression or expanded amino acid tracts also contribute to HDL2 pathogenesis.Methods:Transcripts from the HDL2 locus, and their protein products, were examined in HDL2, HD, and control frontal cortex. The effect of loss of Jph3 was examined in mice with partial or complete loss of Jph3.Results:Bidirectional transcription occurs at the HDL2 locus, although expression of antisense transcripts with expanded CAG repeats is limited. Protein products with expanded amino acid tracts were not detected in HDL2 brain. However, JPH3 transcripts and full‐length JPH3 protein are decreased in HDL2 brain, and Jph3 hemizygous and null mice exhibit abnormal motor function.Interpretation:Our results suggest that the pathogenic mechanism of HDL2 is multifactorial, involving both a toxic gain of function of JPH3 RNA and a toxic loss of JPH3 expression. Ann Neurol 2012;71:245–257
Angiogenin variants in Parkinson disease and amyotrophic lateral sclerosis
Authors: van Es Michael A. , Schelhaas Helenius J. , van Vught Paul W. J. , Ticozzi Nicola , Andersen Peter M. , Groen Ewout J. N. , Schulte Claudia , Blauw Hylke M. , Koppers Max , Diekstra Frank P. , Fumoto Katsumi , LeClerc Ashley Lyn , Keagle Pamela , Bloem Bastiaan R. , Scheffer Hans , van Nuenen Bart F. L. , van Blitterswijk Marka , van Rheenen Wouter , Wills Anne‐Marie , Lowe Patrick P. , Hu Guo‐fu , Yu Wenhao , Kishikawa Hiroko , Wu David , Folkerth Rebecca D. , Mariani Claudio , Goldwurm Stefano , Pezzoli Gianni , Van Damme Philip , Lemmens Robin , Dahlberg Caroline , Birve Anna , Fernández‐Santiago Rubén , Waibel Stefan , Klein Christine , Weber Markus , van der Kooi Anneke J. , de Visser Marianne , Verbaan Dagmar , van Hilten Jacobus J. , Heutink Peter , Hennekam Eric A. M. , Cuppen Edwin , Berg Daniela , Brown Robert H. , Silani Vincenzo , Gasser Thomas , Ludolph Albert C. , Robberecht Wim , Ophoff Roel A. , Veldink Jan H. , Pasterkamp R. Jeroen , de Bakker Paul I. W. , Landers John E. , van de Warrenburg Bart P. , van den Berg Leonard H. 
Published: August 2011

AbstractObjective:Several studies have suggested an increased frequency of variants in the gene encoding angiogenin (ANG) in patients with amyotrophic lateral sclerosis (ALS). Interestingly, a few ALS patients carrying ANG variants also showed signs of Parkinson disease (PD). Furthermore, relatives of ALS patients have an increased risk to develop PD, and the prevalence of concomitant motor neuron disease in PD is higher than expected based on chance occurrence. We therefore investigated whether ANG variants could predispose to both ALS and PD.Methods:We reviewed all previous studies on ANG in ALS and performed sequence experiments on additional samples, which allowed us to analyze data from 6,471 ALS patients and 7,668 controls from 15 centers (13 from Europe and 2 from the USA). We sequenced DNA samples from 3,146 PD patients from 6 centers (5 from Europe and 1 from the USA). Statistical analysis was performed using the variable threshold test, and the Mantel‐Haenszel procedure was used to estimate odds ratios.Results:Analysis of sequence data from 17,258 individuals demonstrated a significantly higher frequency of ANG variants in both ALS and PD patients compared to control subjects (p = 9.3 × 10−6 for ALS and p = 4.3 × 10−5 for PD). The odds ratio for any ANG variant in patients versus controls was 9.2 for ALS and 6.7 for PD.Interpretation:The data from this multicenter study demonstrate that there is a strong association between PD, ALS, and ANG variants. ANG is a genetic link between ALS and PD. ANN NEUROL 2011;70:964–973
Zuverlässige und effiziente Konjugation von Biomolekülen über Huisgen‐Azid‐Alkin‐Cycloadditionen
Authors: Lallana Enrique , Riguera Ricardo , Fernandez‐Megia Eduardo 
Published: August 2011

AbstractDie CuI‐katalysierte Azid‐Alkin‐Cycloaddition (CuAAC) ist ein etabliertes Biokonjugationsverfahren für Proteine, Nucleinsäuren und Polysaccharide. Der Einsatz von Cu führt aber zu einigen Problemen, hauptsächlich in Form von oxidativen Zersetzungsprozessen durch reaktive Sauerstoffspezies sowie einer Kontamination der Probe mit Cu. Dieser Kurzaufsatz stellt die wichtigsten Fortschritte bei der effizienten und schonenden Funktionalisierung von Biomakromolekülen über CuAAC und die Cu‐freie ringspannungsvermittelte Azid‐Alkin‐Cycloaddition (SPAAC) vor.
Polyethylene glycol‐polyethylenimine‐tetrachloroplatinum (IV): A novel conjugate with good abilities of antitumor and gene delivery
Authors: Zhao Lu , Xu Fujian , Chen Huan , Tang Guping , Hu Xiurong 
Published: August 2011

AbstractIt is much importance to develop novel multifunctional delivery systems for the combination therapy of drug and gene. In this work, a novel conjugate, polyethylene glycol‐polyethylenimine‐tetrachloroplatinum (IV) (PEG‐PEI‐Pt), with good abilities of antitumor and gene delivery was proposed by combining PEG (Mw 3400 Da), low molecular weight PEI (Mw 800 Da), and tetrachloroplatinum (IV). The antitumoral and gene transfection activities of PEG‐PEI‐Pt were analyzed in many tumor (A549, A375, HepG‐2, HuH‐7, and B16 cells) and normal (COS‐7 cells) cell lines. Similar to cisplatin (one platinum anticancer drug), PEG‐PEI‐Pt showed much higher sensitivity in tumor cells than in normal cells. More importantly, PEG‐PEI‐Pt had a potential to treat drug‐resistant tumors. Almost no transfection efficiency was observed for PEI (Mw 800 Da) and PEG‐PEI. Very interestingly, PEG‐PEI‐Pt could condense plasmid DNA efficiently, and exhibited good transfection efficiency in B16, HepG‐2, A375 and COS‐7 cells, comparable to even higher than PEI 25 kDa. In addition, PEG‐PEI‐Pt could also effectively deliver siRNA into the cytoplasm of tumor cells. With the good antitumoral and gene delivery abilities, PEG‐PEI‐Pt may have a great potential for combination therapy of drug and gene. © 2011 Wiley Periodicals, Inc. J Appl Polym Sci, 2012
Inclusion complex of cantharidin with β‐cyclodextrin: Preparation, characterization, in vitro and in vivo evaluation
Authors: Dang Yun‐Jie , An Lin‐Na , Hu Chunhui , Zhu Chun‐Yan 
Published: August 2011

AbstractBecause of low aqueous solubility and slow dissolution rate, cantharidin has a low oral bioavailability. Our research aims to prepare the inclusion complex of cantharidin and β‐cyclodextrin (β‐CD) and accomplish characterization, in vitro and in vivo evaluation. CA‐β‐CD inclusion complex was prepared by saturated solution method. The CA was demonstrated by HPLC in vitro experiment and by GC‐MS in vivo experiment. CA‐β‐CD inclusion complex was characterized by differential scanning calorimetry (DSC), X‐ray diffractometry (XRD), and nuclear magnetic resonance (NMR). Through complexation with β‐CD, the solubility of CA in neutral aqueous solution was improved significantly. CA‐β‐CD inclusion complex also shows a significantly improved dissolution rate in comparison with free CA. Comparison of the pharmacokinetics between CA‐β‐CD inclusion complex and free CA was performed in rats. The in vivo results show that CA‐β‐CD inclusion complex has earlier tmax, higher Cmax, and higher bioavailability than free CA after oral dosing. By comparing the AUC0–t of CA and CA‐β‐CD inclusion complex, the relative bioavailability of CA‐β‐CD inclusion complex to free CA was 506.3%, which highlighted the evidence of significantly improved bioavailability of formulation of CA with β‐CD. Thus, this β‐CD‐based drug delivery system should be an effective oral dosage form to improve oral bioavailability of CA. © 2011 Wiley Periodicals, Inc. J Appl Polym Sci, 2012
Femoral Morphology and Femoropelvic Musculoskeletal Anatomy of Humans and Great Apes: A Comparative Virtopsy Study
Authors: Morimoto Naoki , De León Marcia S. Ponce , Nishimura Takeshi , Zollikofer Christoph P.E. 
Published: August 2011

AbstractScheme of virtual dissection (virtopsy). Volume data visualization was performed for CT data of a formalin‐preserved juvenile chimpanzee (id: AIMUZH‐7283) to visualize muscles and skeleton. See Figure 2B and 2C in Morimoto et al., on page 1437, in this issue.
What does the g‐index really measure?
Authors: De Visscher Alex 
Published: August 2011

AbstractIt was argued recently that the g‐index is a measure of a researcher's specific impact (i.e., impact per paper) as much as it is a measure of overall impact. While this is true for the productive “core” of publications, it can be argued that the g‐index does not differ from the square root of the total number of citations in a bibliometrically meaningful way when the entire publication list is considered. The R‐index also has a tendency to follow total impact, leaving only the A‐index as a true measure of specific impact. The main difference between the g‐index and the h‐index is that the former penalizes consistency of impact whereas the latter rewards such consistency. It is concluded that the h‐index is a better bibliometric tool than is the g‐index, and that the square root of the total number of citations is a convenient measure of a researcher's overall impact.
When transparency and collaboration collide: The USA Open Data program
Authors: Peled Alon 
Published: August 2011

AbstractPresident Obama's inaugural flagship Open Data program emphasizes the values of transparency, participation, and collaboration in governmental work. The Open Data performance data analysis, published here for the first time, proposes that most federal agencies have adopted a passive–aggressive attitude toward this program by appearing to cooperate with the program while in fact effectively ignoring it. The analysis further suggests that a tiny group of agencies are the only “real players” in the Data.gov web arena. This research highlights the contradiction between Open Data's transparency goal (“All data must be freed”) and federal agencies' goal of collaborating with each other through data trade. The research also suggests that agencies comprehended that Open Data is likely to exacerbate three critical, back‐office data‐integration problems: inclusion, confusion, and diffusion. The article concludes with a proposal to develop an alternative Federal Information Marketplace (FIM) to incentivize agencies to improve data sharing.
Sulfonic and Phosphonic Acid and Bifunctional Organic–Inorganic Hybrid Membranes and Their Proton Conduction Properties
Authors: Sel Ozlem , Azais Thierry , Maréchal Manuel , Gébel Gérard , Laberty‐Robert Christel , Sanchez Clément 
Published: August 2011

AbstractHybrid organic–inorganic approaches are used for the synthesis of bifunctional proton exchange membrane fuel cell (PEMFC) membranes owing to their ability to combine the properties of a functionalized inorganic network and an organic thermostable polymer. We report the synthesis of both sulfonic and phosphonic acid functionalized mesostructured silica networks into a poly(vinylidenefluoride‐co‐hexafluoropropylene) (poly(VDF‐co‐HFP) copolymer. These membranes, containing different amounts of phosphonic acid and sulfonic acid groups, have been characterized using FTIR and NMR spectroscopy, SA‐XRD, SAXS, and electrochemical techniques. The proton conductivity of the bifunctional hybrid membranes depends strongly on hydration, increasing by two orders of magnitude over the relative humidity (RH) range of 20 to 100 %, up to a maximum of 0.031 S cm−1 at 60 °C and 100 % RH. This value is interesting as only half of the membrane conducts protons. This approach allows the synthesis of a porous SiO2 network with two different functions, having SO3H and PO3H2 embedded in a thermostable polymer matrix.
How ubiquitination regulates the TGF‐β signalling pathway: New insights and new players
Authors: Soond Surinder M. , Chantry Andrew 
Published: August 2011

AbstractUbiquitination of protein species in regulating signal transduction pathways is universally accepted as of fundamental importance for normal development, and defects in this process have been implicated in the progression of many human diseases. One pathway that has received much attention in this context is transforming growth factor‐beta (TGF‐β) signalling, particularly during the regulation of epithelial‐mesenchymal transition (EMT) and tumour progression. While E3‐ubiquitin ligases offer themselves as potential therapeutic targets, much remains to be unveiled regarding mechanisms that culminate in their regulation. With this in mind, the focus of this review highlights the regulation of the ubiquitination pathway and the significance of a recently described group of NEDD4 E3‐ubiquitin ligase isoforms in the context of TGF‐β pathway regulation. Moreover, we now broaden these observations to incorporate a growing number of protein isoforms within the ubiquitin ligase superfamily as a whole, and discuss their relevance in defining a new ‘iso‐ubiquitinome’.
Laccase mediator systems for eco‐friendly production of medium‐density fiberboard (MDF) on a pilot scale: Physicochemical analysis of the reaction mechanism
Authors: Euring Markus , Rühl Martin , Ritter Nina , Kües Ursula , Kharazipour Alireza 
Published: August 2011

AbstractIncreasing prices of petrochemical resins and possible harmful formaldehyde emissions from conventionally produced wood composites have resulted in increased interest in enzymatic binder systems as environmentally friendly alternatives for gluing lignocellulosic products. In this study, laccase mediator systems (LMSs) were used to activate lignin on wood fiber surfaces in the pilot‐scale production of medium‐density fiberboard (MDF) using a dry process. Three different mediators were applied: 4‐hydroxybenzoic acid (HBA), 1‐hydroxybenzotriazole (HBT), and acetosyringone (AS) of which HBA performed best. The mechanical properties of the manufactured boards produced with thermomechanical pulp (TMP) fibers, laccase, and HBA fulfilled all required European standards for wood‐based panels. Oxygen consumption rates of the different LMSs and 13C NMR spectroscopy results for treated TMP fibers were obtained for qualitative and quantitative analysis of lignin activation. The results show that reactions were most effective within the first 30 min of incubation. Oxygen consumption was fastest and highest for the LMS using HBA. 13C NMR spectroscopy indicated the highest decrease of aromatic groups in the wood fiber lignin with this LMS. The data correlated well with the quality of the MDF. The required enzymatic reaction times allowed direct integration of the LMS into standard MDF production techniques. The results indicate that application of LMSs has a high potential for environmentally friendly MDF production.
Data management as bibliography
Authors: Buckland Michael 
Published: August 2011

AbstractEditor's SummaryA critical element in the products of research projects is the data set, largely treated as a marginal appendage to the written record. With the requirement for data management plans in grant proposals to the National Science Foundation and National Institutes of Health, the issue of managing research data sets is gaining attention. Sharing primary data enables a greater return on the original investment, expanded discovery and fertilization of ideas across disciplines. The major impediment is lack of an infrastructure to archive non‐textual data sets, in addition to hurdles including locating data, deterioration, format standardization, permission for use and suitability. It is essential that institutional policies recognize data sets as a key product of intellectual work worthy of inclusion in a bibliography. This must be followed by action to promote their preservation, metadata enrichment, cross‐lingual interoperability, geographic and temporal coding and data provenance and to contend with changes over time.
Crystal Structures of Novel Allosteric Peptide Inhibitors of HIV Integrase Identify New Interactions at the LEDGF Binding Site
Authors: Rhodes David I. , Peat Thomas S. , Vandegraaff Nick , Jeevarajah Dharshini , Newman Janet , Martyn John , Coates Jonathan A. V. , Ede Nicholas J. , Rea Philip , Deadman John J. 
Published: August 2011

AbstractAn optimised method of solution cyclisation gave us access to a series of peptides including SLKIDNLD (2). We investigated the crystallographic complexes of the HIV integrase (HIV‐IN) catalytic core domain with 13 of the peptides and identified multiple interactions at the binding site, including hydrogen bonds with residues Thr125 and Gln95, that have not previously been described as being accessible within the binding site. We show that the peptides inhibit the interaction of lens epithelium‐derived growth factor (LEDGF) with HIV‐IN in a proximity AlphaScreen assay and in an assay for the LEDGF enhancement of HIV‐IN strand transfer. The interactions identified represent a potential framework for the development of new HIV‐IN inhibitors.
New Promising Iridium Catalyst for Toluene Hydrogenation
Authors: Zieliński Michał , Pietrowski Mariusz , Wojciechowska Maria 
Published: August 2011

AbstractThe use of an iridium active phase and magnesium fluoride as a support allowed us to obtain new catalysts of high activities in the hydrogenation of toluene. The Ir/MgF2catalyst was four times more active than the iridium system supported on commercial Al2O3. The nature of iridium precursors [H2IrCl6or Ir4(CO)12] and catalyst pretreatment conditions (reduction or oxidation/reduction) appeared to have a clear effect on the activity of Ir/MgF2catalysts. For the catalysts obtained from iridium carbonyl, the highest activity was obtained after direct reduction of the catalysts, whereas for the catalysts obtained from hexachloroiridium acid, the highest activity was obtained after reduction of the preliminary oxidized catalysts.
Water‐ and Acid‐Mediated Excited‐State Intramolecular Proton Transfer and Decarboxylation Reactions of Ketoprofen in Water‐Rich and Acidic Aqueous Solutions
Authors: Li Ming‐De , Yeung Chi Shun , Guan Xiangguo , Ma Jiani , Li Wen , Ma Chensheng , Phillips David Lee 
Published: August 2011

AbstractWe present an investigation of the decarboxylation reaction of ketoprofen (KP) induced by triplet excited‐state intramolecular proton transfer in water‐rich and acidic solutions. Nanosecond time‐resolved resonance Raman spectroscopy results show that the decarboxylation reaction is facile in aqueous solutions with high water ratios (water/acetonitrile≥50 %) or acidic solutions with moderate and strong acid concentration. These experimental results are consistent with results from density functional theory calculations in which 1) the activation energy barriers for the triplet‐state intramolecular proton transfer and associated decarboxylation process become lower when more water molecules (from one up to four molecules) are involved in the reaction system and 2) perchloric acid, sulfuric acid, and hydrochloric acid can shuttle a proton from the carboxyl to carbonyl group through an initial intramolecular proton transfer of the triplet excited state, which facilitates the cleavage of the CC bond, thus leading to the decarboxylation reaction of triplet state KP. During the decarboxylation process, the water molecules and acid molecules may act as bridges to mediate intramolecular proton transfer for the triplet state KP when KP is irradiated by ultraviolet light in water‐rich or acidic aqueous solutions and subsequently it generates a triplet‐protonated carbanion biradical species. The faster generation of triplet‐protonated carbanion biradical in acidic solutions than in water‐rich solutions with a high water ratio is also supported by the lower activation energy barrier calculated for the acid‐mediated reactions versus those of water‐molecule‐assisted reactions.
High‐Separation Performance of Chromatographic Capillaries Coated with MOF‐5 by the Controlled SBU Approach
Authors: Münch Alexander S. , Seidel Jürgen , Obst Anja , Weber Edwin , Mertens Florian O. R. L. 
Published: August 2011

AbstractRecently developed MOF surface‐coating techniques, the controlled SBU approach (CSA) for the generation of MOF‐5, and the use of self‐assembled monolayers have been combined to generate a wall‐bonded, crosslinked stationary phase for gas chromatographic capillary columns displaying excellent performance in the separation of natural gas components. The chromatographic performance of this new type of column has been compared to the state‐of‐the‐art solution for this separation problem, namely a coated silica column of the porous layer open tubular (PLOT) type. Chromatographic parameters such as separation, resolution, and tailing factors, as well as plate numbers and heights in the case of isothermal operation, have been determined. Kinetic and thermodynamic parameters characterizing the analyte–stationary phase interaction have been determined for various C1–C4 analytes.
Simultaneous Detection of Intracellular Tumor mRNA with Bi‐Color Imaging Based on a Gold Nanoparticle/Molecular Beacon
Authors: Qiao Guangming , Gao Yuan , Li Na , Yu Zhengze , Zhuo Linhai , Tang Bo 
Published: August 2011

AbstractThe successful treatment of most cancers depends on early detection. Tumor mRNA as a specific marker provides new avenues to monitor tumor progression in the early stages and assesses response to treatment. However, single tumor mRNA testing usually yields “false positive” results because cancer is associated with multiple tumor mRNA. It is indispensable to develop simple and effective approaches for the detection of multiple tumor mRNA. In this study, we used a combination of tumor‐specific mRNA markers to avoid the inherent limitations associated with the single‐marker technique. A gold nanoparticle (AuNP) was assembled with a bi‐molecular beacon (bi‐MB), and termed AuNP/bi‐MB, which simultaneously targeted to two types of tumor mRNA in breast cancer cells. This imaging agent could prevent effectively false positive results and provide comprehensive and dependable information for the early detection of cancer. It would be beneficial to identify the stage of tumor progression and assess treatment decisions with the real‐time detection of the relative expression levels of tumor mRNA in cancer cells. This strategy would offer an appealing approach toward the early detection of cancer by using multianalysis of tumor mRNA.