bluuebunny/crossref_metadata_2025_split huggingface dataset:
De novo LMNA mutations cause a new form of congenital muscular dystrophy
Authors: Quijano‐Roy Susana , Mbieleu Blaise , Bönnemann Carsten G. , Jeannet Pierre‐Yves , Colomer Jaume , Clarke Nigel F. , Cuisset Jean‐Marie , Roper Helen , De Meirleir Linda , D'Amico Adele , Ben Yaou Rabah , Nascimento Andrés , Barois Annie , Demay Laurence , Bertini Enrico , Ferreiro Ana , Sewry Caroline A. , Romero Norma B. , Ryan Monique , Muntoni Francesco , Guicheney Pascale , Richard Pascale , Bonne Gisèle , Estournet Brigitte 
Published: June 2008

AbstractObjectiveTo describe a new entity of congenital muscular dystrophies caused by de novo LMNA mutations.MethodsFifteen patients presenting with a myopathy of onset in the first year of life were subjected to neurological and genetic evaluation. Histopathological and immunohistochemical analyses were performed for all patients.ResultsThe 15 patients presented with muscle weakness in the first year of life, and all had de novo heterozygous LMNA mutations. Three of them had severe early‐onset disease, no motor development, and the rest experienced development of a “dropped head” syndrome phenotype. Despite variable severity, there was a consistent clinical pattern. Patients typically presented with selective axial weakness and wasting of the cervicoaxial muscles. Limb involvement was predominantly proximal in upper extremities and distal in lower extremities. Talipes feet and a rigid spine with thoracic lordosis developed early. Proximal contractures appeared later, most often in lower limbs, sparing the elbows. Ten children required ventilatory support, three continuously through tracheotomy. Cardiac arrhythmias were observed in four of the oldest patients but were symptomatic only in one. Creatine kinase levels were mild to moderately increased. Muscle biopsies showed dystrophic changes in nine children and nonspecific myopathic changes in the remaining. Markedly atrophic fibers were common, most often type 1, and a few patients showed positive inflammatory markers.InterpretationThe LMNA mutations identified appear to correlate with a relatively severe phenotype. Our results further broaden the spectrum of laminopathies and define a new disease entity that we suggest is best classified as a congenital muscular dystrophy (LMNA‐related congenital muscular dystrophy, or L‐CMD). Ann Neurol 2008.
Flocking in multi‐agent systems with multiple virtual leaders
Authors: Su Housheng , Wang Xiaofan , Yang Wen 
Published: June 2008

AbstractMany tracking problems in flocking are related to a single (virtual) leader with a constant velocity. In this paper, we investigate the problem of controlling a group of mobile autonomous agents to track multiple virtual leaders with varying velocities in the sense that agents with the same virtual leader attain the same velocity and track the corresponding leader. We propose a provably‐stable flocking algorithm. Moreover, we show that the position and velocity of the center of the mass of all agents will exponentially converge to weighted average position and velocity of the virtual leaders. Numerical simulations are worked out to illustrate theoretical results. Copyright © 2008 John Wiley and Sons Asia Pte Ltd and Chinese Automatic Control Society
Distributed control of multi‐agent systems with second‐order agent dynamics and delay‐dependent communications
Authors: Lin Peng , Jia Yingmin , Du Junping , Yuan Shiying 
Published: June 2008

AbstractThis paper investigates distributed consensus problems in networks of second‐order agents with time‐delay and switching topology. A delayed linear consensus protocol is introduced and the corresponding convergence analysis is provided as well. Some sufficient conditions are given which ensure the agreement of all agents under arbitrary switching of the network topology in presence of time‐delay. All obtained conditions are expressed as linear matrix inequalities. Copyright © 2008 John Wiley and Sons Asia Pte Ltd and Chinese Automatic Control Society
Stereoselective analysis of carvedilol in human plasma and urine using HPLC after chiral derivatization
Authors: Peccinini Rosângela G. , Ximenes Valdecir F. , Cesarino Evandro J. , Lanchote Vera L. 
Published: June 2008

AbstractAn enantioselective high‐performance liquid chromatographic method for the analysis of carvedilol in plasma and urine was developed and validated using (‐)‐menthyl chloroformate (MCF) as a derivatizing reagent. Chloroform was used for extraction, and analysis was performed by HPLC on a C18 column with a fluorescence detector. The quantitation limit was 0.25 ng/ml for S(−)‐carvedilol in plasma and 0.5 ng/ml for R(+)‐carvedilol in plasma and for both enantiomers in urine. The method was applied to the study of enantioselectivity in the pharmacokinetics of carvedilol administered in a multiple dose regimen (25 mg/12 h) to a hypertensive elderly female patient. The data obtained demonstrated highest plasma levels for the R(+)‐carvedilol (AUC 75.64 vs 37.29 ng/ml). The enantiomeric ratio R(+)/S(−) was 2.03 for plasma and 1.49 for urine (Ae0–12 17.4 vs 11.7 µg). Copyright © 2008 John Wiley & Sons, Ltd.
Influence of green and black tea on folic acid pharmacokinetics in healthy volunteers: potential risk of diminished folic acid bioavailability
Authors: Alemdaroglu N. Ceren , Dietz Ulrich , Wolffram Siegfried , Spahn‐Langguth Hildegard , Langguth Peter 
Published: June 2008

AbstractPrevious in vitro studies using Caco‐2 cell monolayers suggested a possible interaction between green and black tea and folic acid at the level of intestinal absorption. The main purpose of the present study was to investigate a possible pharmacokinetic interaction between tea and folic acid in healthy volunteers. In an open‐labeled randomized cross‐over study, the pharmacokinetic interaction between tea and folic acid (0.4 mg and 5 mg) was investigated in healthy volunteers. Water was used as the reference drink. Subjects ingested 0.4 mg folic acid tablets with water, green or black tea (0.3 g extract/250 ml) or 5 mg folic acid tablets with water or green tea (0.3 g extract/250 ml). Blood samples were collected over a period of 8 h. Serum folate analysis was carried out by a competitive immunoassay which uses direct chemiluminescent technology. At the 0.4 mg folic acid dose, green and black tea reduced the mean Cmax of serum folate by 39.2% and 38.6%, and the mean AUC0 → ∞ by 26.6% and 17.9%, respectively. At the 5 mg folic acid dose, the mean Cmax of serum folate was reduced by 27.4% and the mean AUC0 → ∞ was decreased significantly by 39.9% by the co‐application of green tea. The present results suggest an in vivo interaction between tea and folic acid with even low concentrations of green and black tea extracts yielding decreased bioavailabilities of folic acid. Copyright © 2008 John Wiley & Sons, Ltd.
Reaction mechanism of surfactant‐sensitized chemiluminescence of bis(2,4,6‐trichlorophyenyl) oxalate and hydrogen peroxide induced by gold nanoparticles
Authors: Liang Shu‐Xuan , Li Haifang , Lin Jin‐Ming 
Published: June 2008

AbstractThe chemiluminescence (CL) of bis(2,4,6‐trichlorophyenyl) oxalate with hydrogen peroxide in the present of cationic surfactant and gold nanoparticles was studied. The CL emission was obviously enhanced in the presence of surfactant at a suitable concentration, with a synergetic catalysis effect exhibited. Different sizes of gold nanoparticles (15 and 50 nm) showed different effects on CL intensity. Mechanisms of the CL reaction and sensitization effect are discussed. Copyright © 2008 John Wiley & Sons, Ltd.
Prospective study of bone scintigraphy as a staging investigation for oesophageal carcinoma
Authors: Jennings N A , Griffin S M , Lamb P J , Preston S , Richardson D , Karat D , Hayes N 
Published: June 2008

Abstract

Background
About 10 per cent of patients undergoing radical oesophagectomy for transmural (T3) carcinoma with lymph node involvement (N1) develop symptomatic bone metastases within 12 months of surgery. The aim of this study was to evaluate the introduction of targeted preoperative bone scintigraphy.

Methods
Of 790 patients with oesophageal carcinoma staged between December 2000 and December 2004, 189 were eligible for potentially curative treatment. 99mTc-labelled hydroxymethylene diphosphonate bone scintigraphy was performed in those with stage T3 N1 disease (identified by computed tomography and endoscopic ultrasonography) who were suitable for radical treatment.

Results
A total of 115 patients had bone scintigraphy. The histological diagnosis was adenocarcinoma in 82 patients and squamous cell carcinoma in 33. Bone scintigraphy was normal or showed degenerative changes in 93 patients, and abnormal requiring further investigation in 22. Plain radiography, magnetic resonance imaging and biopsy confirmed the presence of bone metastases in 11 patients (9·6 per cent).

Conclusion
Bone is frequently the first site of identifiable distant metastatic spread, and bone scintigraphy is recommended to exclude metastatic disease before radical treatment of advanced oesophageal carcinoma.
Asymmetric Three‐Component Strecker Reactions Catalyzed by trans‐4‐Hydroxy‐L‐proline‐Derived N,N′‐Dioxides
Authors: Wen Yuehong , Gao Bo , Fu Yingzi , Dong Shunxi , Liu Xiaohua , Feng Xiaoming 
Published: June 2008

AbstractNovel trans‐4‐hydroxy‐L‐proline‐derived N,N′‐dioxides have been developed and used as efficient organocatalysts for the one‐pot three‐component Strecker reaction with an aldehyde, (1,1‐diphenyl)methylamine, and TMSCN. Both aromatic and aliphatic aldehydes were found to be suitable substrates. The corresponding α‐amino nitriles were obtained in high yields with up to 95 % ee (ee=enantiomeric excess) under mild conditions. Optically pure products could be obtained after a single recrystallization. The catalyst can be easily prepared from trans‐4‐hydroxy‐L‐proline and a diamine in three steps. Based on the experimental results and the observed absolute configurations of the products, a possible transition state has been proposed to explain the origin of the asymmetric induction.
Entorhinal cortex of the monkey: IV. Topographical and laminar organization of cortical afferents
Authors: Insausti Ricardo , Amaral David G. 
Published: June 2008

AbstractThe nonhuman primate entorhinal cortex is the primary interface for information flow between the neocortex and the hippocampal formation. Based on previous retrograde tracer studies, neocortical afferents to the macaque monkey entorhinal cortex originate largely in polysensory cortical association areas. However, the topographical and laminar distributions of cortical inputs to the entorhinal cortex have not yet been comprehensively described. The present study examines the regional and laminar termination of projections within the entorhinal cortex arising from different cortical areas. The study is based on a library of 51 3H‐amino acid injections that involve most of the afferent regions of the entorhinal cortex. The range of termination patterns was broad. Some areas, such as the medial portion of orbitofrontal area 13 and parahippocampal areas TF and TH, project widely within the entorhinal cortex. Other areas have a more focal and regionally selective termination. The lateral orbitofrontal, insular, anterior cingulate, and perirhinal cortices, for example, project only to rostral levels of the entorhinal cortex. The upper bank of the superior temporal sulcus projects mainly to intermediate levels of the entorhinal cortex, and the parietal and retrosplenial cortices project to caudal levels. The projections from some of these cortical regions preferentially terminate in the superficial layers (I–III) of the entorhinal cortex, whereas others project more heavily to the deep layers (V–VI). Thus, some of the cortical inputs may be more influential on the cortically directed outputs of the hippocampal formation or on gating neocortical information flow into the other fields of the hippocampal formation rather than contributing to the perforant path inputs to other hippocampal fields. J. Comp. Neurol. 509:608–641, 2008. © 2008 Wiley‐Liss, Inc.
Robust tracking control for robot systems with discontinuous uncertainty: an approach based on Filippov's framework
Authors: Nakakuki Takashi , Shen Tielong , Tamura Katsutoshi 
Published: June 2008

AbstractIn this paper, the robust tracking control problem for a rigid robot with discontinuous uncertainty is considered. The controller design approach is based on Filippov's framework and generalized Lyapunov stability theory. It is shown that no Lipschitz continuous controller to stabilize the error system asymptotically exists because of the existence of nontrivial equilibrium set including the origin. A robust controller which makes the error system asymptotically stable is derived in Filippov's framework. © 2008 Wiley Periodicals, Inc. Electr Eng Jpn, 164(4): 53–61, 2008; Published online in Wiley InterScience (www. interscience.wiley.com). DOI 10.1002/eej.20303
Potentiality of a new minimum‐order back‐EMF state observer taking acceleration into account for sensorless vector control of permanent‐magnet synchronous motors
Authors: Shinnaka Shinji , Saito Yoji 
Published: June 2008

AbstractThis paper proposes new sensorless vector control methods for permanent‐magnet synchronous motors (PMSMs), which are based on a new minimum‐order back‐EMF state observer taking acceleration into account, and examines potentiality of the improved back‐EMF observer through experiments. Conventional sensorless vector control methods for PMSMs using an estimate of back‐EMF are established under the assumption of constant speed or zero acceleration, and consequently cannot operate properly at modes requiring rapid speed change especially in low speed region. On the other hand, the proposed back‐EMF observer has the following features: (1) it is a new back‐EMF state observer taking acceleration into account and requiring no additional approximation to motor mathematical model; (2) it is a minimum‐order state observer; (3) it utilizes motor parameters in the simplest manner; (4) it can be applied to both salient‐pole and non‐salient‐pole PMSMs; (5) it can be realized in both rotor and stator reference frames. Detailed designs and analyses for the improved state observer and the sensorless vector control systems in both rotor and stator reference frames are given. In relation to the sensorless vector control systems, this paper newly proposes a double‐integral‐type PLL method and an integral‐feedback‐type acceleration/speed estimation method. Their potentialities are examined through experiments. © 2008 Wiley Periodicals, Inc. Electr Eng Jpn, 164(4): 78–92, 2008; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/ eej.20630
Magnetic properties of melt‐spun Pr(Nd)‐Fe‐Co‐Nb‐Cu‐Si‐B system ribbons and their bonded magnets
Authors: Yamamoto Hiroshi , Shinagawa Hiroki 
Published: June 2008

AbstractMelt‐spun ribbons of Pr‐Fe‐Co‐Nb‐Cu‐Si‐B system alloys were prepared by a single roller rapid‐quenching method. The effects of composition, wheel velocity, and heat treatment on their magnetic properties were investigated. The optimum preparation conditions of compounds were as follows: composition Pr9Fe68.5Co10Nb1CuSi0.5B10.5; wheel velocity 12.5 m/s; heat treatment 625°C for 5 min. The magnetic properties obtained were (BH)max=121.8kJ/m3,Jr=0.90 T, HcJ=898.7kA/m, and HcB=509.0kA/m. The temperature coefficients of Jr and HcJ of the ribbons of Pr9Fe68.5Co10Nb1Cu0.5Si0.5B10.5 alloy were α(Jr)ave=–0.034%/°C and α(HcJ)ave=–0.290%/°C, respectively. The value of (BH)max for isotropic Pr9Fe68.5Co10Nb1Cu0.5Si0.5B10.5 bonded magnets prepared by using ribbons annealed at 625°C for 5 min was 84.4kJ/m3. The irreversible loss of the bonded magnets was –2.74% at 125°C. © 2008 Wiley Periodicals, Inc. Electr Eng Jpn, 164(4): 1–7, 2008; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/eej.20638
Development of satellite glia in mouse sympathetic ganglia: GDNF and GFRα1 are not essential
Authors: Callahan Thomas , Young Heather M. , Anderson Richard B. , Enomoto Hideki , Anderson Colin R. 
Published: June 2008

AbstractThe phenotypic development of satellite cells in mouse sympathetic ganglia was examined by localizing the transcription factors, Sox10 and Phox2b, the neuronal marker, tyrosine hydroxylase (TH), and brain‐derived fatty acid binding protein (B‐FABP), which identifies glial precursors and mature glia. In E10.5 mice, most cells in the sympathetic chain expressed both Sox10 and Phox2b, with a minority of cells expressing Sox10 only or Phox2b only. In E11.5 mice, the majority of cells expressed Sox10 only or Phox2b only. B‐FABP was colocalized with Sox10 in satellite glial precursors, which were located on the periphery of the ganglion. There was no overlap between B‐FABP and Phox2b or B‐FABP and TH. During subsequent development, the number of B‐FABP+ cells increased and they became more common deep within the ganglion. In E12.5 and E18.5 mice, there was no overlap between Sox10 and Phox2b, and 98% of Sox10 cells were also B‐FABP+. Satellite glial precursors in E11.5–E15.5 mice also expressed the GDNF‐binding molecule, GFRα1. B‐FABP immunoreactive cells did not express Ret or NCAM, two potential signaling molecules for GDNF/GFRα1. In E12.5 and E18.5 mice lacking GFRα1 or GDNF, the development of B‐FABP immunoreactive satellite cells was normal, and hence neither GDNF or GFRα1 are essential for the development of satellite glia in sympathetic ganglia. © 2008 Wiley‐Liss, Inc.
Nucleotide‐induced Ca2+signaling in sustentacular supporting cells of the olfactory epithelium
Authors: Hassenklöver Thomas , Kurtanska Silvia , Bartoszek Ilonka , Junek Stephan , Schild Detlev , Manzini Ivan 
Published: June 2008

AbstractExtracellular purines and pyrimidines are important signaling molecules acting via purinergic cell‐surface receptors in neurons, glia, and glia‐like cells such as sustentacular supporting cells (SCs) of the olfactory epithelium (OE). Here, we thoroughly characterize ATP‐induced responses in SCs of the OE using functional Ca2+imaging. The initial ATP‐induced increase of the intracellular Ca2+concentration [Ca2+]ialways occurred in the apical part of SCs and subsequently propagated toward the basal lamina, indicating the occurrence of purinergic receptors in the apical part of SCs. The mean propagation velocity of the Ca2+signal within SCs was 17.10 ± 1.02 μm/s. ATP evoked increases in [Ca2+]iin both the presence and absence of extracellular Ca2+. Depletion of the intracellular Ca2+stores abolished the responses. This shows that the ATP‐induced [Ca2+]iincreases were in large part, if not entirely, due to the activation of G protein‐coupled receptors followed by Ca2+mobilization from intracellular stores, suggesting an involvement of P2Y receptors. The order of potency of the applied purinergic agonists was UTP > ATP > ATPγS (with all others being only weakly active or inactive). The ATP‐induced [Ca2+]iincreases could be reduced by the purinergic antagonists PPADS and RB2, but not by suramin. Our findings suggest that extracellular nucleotides in the OE activate SCs via P2Y2/P2Y4‐like receptors and initiate a characteristic intraepithelial Ca2+wave. © 2008 Wiley‐Liss, Inc.
E‐cadherin expression in postnatal Schwann cells is regulated by the cAMP‐dependent protein kinase a pathway
Authors: Crawford Audrita T. , Desai Darshan , Gokina Pradeepa , Basak Sayantani , Kim Haesun A. 
Published: June 2008

AbstractExpression of E‐cadherin in the peripheral nervous system is a highly regulated process that appears postnatally in concert with the development of myelinating Schwann cell lineage. As a major component of autotypic junctions, E‐cadherin plays an important role in maintaining the structural integrity of noncompact myelin regions. In vivo, the appearance of E‐cadherin in postnatal Schwann cell is accompanied by the disappearance of N‐cadherin, suggesting reciprocal regulation of the two cadherins during Schwann cell development. The molecular signal that regulates the cadherin switch in Schwann cell is unclear. Using a neuron‐Schwann cell co‐culture system, here we show that E‐cadherin expression is induced by components on the axonal membrane. We also show that the axonal effect is mediated through cAMP‐dependent protein kinase A (cAMP‐PKA) activation in the Schwann cell: (1) inhibition of cAMP‐PKA blocks axon‐induced E‐cadherin expression and (2) cAMP elevation in the Schwann cell is sufficient to induce E‐cadherin expression. In addition, cAMP‐dependent E‐cadherin expression is promoted by contact between adjacent Schwann cell membranes, suggesting its role in autotypic junction formation during myelination. Furthermore, cAMP‐induced E‐cadherin expression is accompanied by suppression of N‐cadherin expression. Therefore, we propose that axon‐dependent activation of cAMP‐PKA serves as a signal that promotes cadherin switch during postnatal development of Schwann cells. © 2008 Wiley‐Liss, Inc.
Eight new bispyridinium oximes in comparison with the conventional oximes pralidoxime and obidoxime: in vivo efficacy to protect from diisopropylfluorophosphate toxicity
Authors: Lorke D. E. , Nurulain S. M. , Hasan M. Y. , Kuča K. , Musilek K. , Petroianu G. A. 
Published: June 2008

AbstractIn search for more efficacious reactivators of acetylcholinesterase (AChE) inhibited by organophosphorus compounds, experimental K‐oximes have been synthesized which show good in vitro efficacy. However, AChE inhibition by oximes themselves (as quantified by their intrinsic IC50) is the major cause of oxime toxicity and the dose‐limiting factor. To assess K‐oxime efficacy in vivo, the extent of protection from mortality induced by diisopropylfluorophosphate (DFP) was quantified by Cox survival analysis and compared with that of the clinically available oximes. Oximes were administered in an equitoxic dosage, i.e. half the LD01. Best protection was conferred by K‐27, reducing the relative risk of death (RR) to 16% of control RR (P ≤ 0.05), which was statistically significantly better (P ≤ 0.05) than all other tested oximes, except obidoxime, K‐53 and K‐75. The efficacy of obidoxime (RR = 0.19), K‐48 (RR = 0.28), K‐53 (RR = 0.22), K‐74 (RR = 0.38) and K‐75 (RR = 0.29) was significantly (P ≤ 0.05) better than that of 2‐PAM (RR = 0.62) and K‐113 (RR = 0.73). No significant protective effect was observed for K‐107 and K‐108. Our LD50 data show that K‐107, K‐108 and K‐113 (which strongly inhibit AChE in vitro) are in vivo markedly more toxic than all other oximes tested and can therefore only be safely administered at a low dosage which is insufficient to protect from DFP‐induced mortality. Dosage calculations based on in vitro IC50 measurements may therefore in future replace in vivo LD50 determinations, thereby reducing the number of animals required. Copyright © 2008 John Wiley & Sons, Ltd.