bluuebunny/crossref_metadata_2025_split huggingface dataset:
Coexistent autoimmune autonomic ganglionopathy and myasthenia gravis associated with non–small‐cell lung cancer
Authors: Peltier Amanda C. , Black Bonnie K. , Raj Satish R. , Donofrio Peter , Robertson David , Biaggioni Italo 
Published: October 2009

AbstractWe report the case of a 55‐year‐old man with non–small‐cell lung cancer who underwent radiation, chemotherapy with carbotaxol and paclitaxel, and left upper lobe removal 2 years prior to evaluation. He was referred for disabling orthostatic hypotension (113/69 mm Hg supine and 66/47 mm Hg standing after 10 minutes) without a compensatory heart rate increase (57 to 59 beats per minute), fatigue, and constipation with episodes of ileus. Clinical examination showed mild ptosis bilaterally, fatiguable neck flexor weakness, and hip flexor weakness. Blood pressure response to Valsalva maneuver was abnormal with an absence of phase 4 overshoot and a Valsalva heart rate ratio of 1.04. Plasma norepinephrine level was low (79 pg/ml supine, 330 pg/ml standing). Single‐fiber electromyography of the right extensor digitorum communis revealed normal mean consecutive difference (jitter) but several pairs exceeded a jitter of 100 μs. Antibodies against muscle acetylcholine receptor [(AChR) 0.66 nmol/L, normal <0.02 nmol/L] and ganglionic AChR (0.34 nmol/L, normal <0.02 nmol/L) were present. Treatment with plasma exchange normalized responses to standing posture (105/68 supine to 118/82 mm Hg standing, 66 to 79 beats per minute), to Valsalva (normal blood pressure overshoot, hazard ratio 1.47), norepinephrine (194 pg/ml supine, 763 pg/ml standing), and jitter measurements. We conclude that autoimmune autonomic ganglionopathy and myasthenia gravis can coexist and suggest that the latter should be excluded in patients with autoimmune autonomic ganglionopathy who complain of fatigue that shows improvement with non‐supine rest. Muscle Nerve, 2009
Clinical findings in MuSK‐antibody positive myasthenia gravis: A U.S. experience
Authors: Pasnoor Mamatha , Wolfe Gil I. , Nations Sharon , Trivedi Jaya , Barohn Richard J. , Herbelin Laura , McVey April , Dimachkie Mazen , Kissel John , Walsh Ronan , Amato Anthony , Mozaffar Tahseen , Hungs Marcel , Chui Luis , Goldstein Jonathan , Novella Steven , Burns Ted , Phillips Lawrence , Claussen Gwendolyn , Young Angela , Bertorini Tulio , Oh Shin 
Published: October 2009

AbstractWe performed a retrospective chart review on 53 muscle‐specific kinase antibody (MuSK‐Ab)‐positive myasthenia gravis (MG) patients at nine university‐based centers in the U.S. Of these, 66% were Caucasian, 85% were women, and age of onset was 9–79 years. Twenty‐seven patients were nonresponsive to anticholinesterase therapy. Myasthenia Gravis Foundation of America improvement status was achieved in 53% patients on corticosteroids, 51% with plasma exchange, and in 20% on intravenous immunoglobulin (IVIG). Thymectomy was beneficial in 7/18 patients at 3 years. Long‐term (≥3 years) outcome was very favorable in 58% of patients who achieved remission and/or minimal manifestation status. Overall, 73% improved. There was one MG‐related death. This survey reinforces several cardinal features of MuSK‐Ab‐positive MG, including prominent bulbar involvement and anticholinesterase nonresponsiveness. Facial or tongue atrophy was rare. Most patients respond favorably to immunotherapy. The best clinical response was to corticosteroids and plasma exchange, and the poorest response was to IVIG. Long‐term outcome is favorable in about 60% of cases. Muscle Nerve, 2009
Multiscale modeling of impact on heterogeneous viscoelastic solids containing evolving microcracks
Authors: Souza Flavio V. , Allen David H. 
Published: October 2009

AbstractMultiscale computational techniques play a major role in solving problems related to viscoelastic composites due to the complexities inherent to these materials. In this paper, a numerical procedure for multiscale modeling of impact on heterogeneous viscoelastic solids containing evolving microcracks is proposed in which the (global scale) homogenized viscoelastic incremental constitutive equations have the same form as the local‐scale viscoelastic incremental constitutive equations, but the homogenized tangent constitutive tensor and the homogenized incremental history‐dependent stress tensor at the global scale depend on the amount of damage accumulated at the local scale. Furthermore, the developed technique allows the computation of the full anisotropic incremental constitutive tensor of viscoelastic solids containing evolving cracks (and other kinds of heterogeneities) by solving the micromechanical problem only once at each material point and each time step. The procedure is basically developed by relating the local‐scale displacement field to the global‐scale strain tensor and using first‐order homogenization techniques. The finite element formulation is developed and some example problems are presented in order to verify the approach and demonstrate the model capabilities. Copyright © 2009 John Wiley & Sons, Ltd.
Non‐coding RNAs: regulators of disease
Authors: Taft Ryan J , Pang Ken C , Mercer Timothy R , Dinger Marcel , Mattick John S 
Published: October 2009

AbstractFor 50 years the term ‘gene’ has been synonymous with regions of the genome encoding mRNAs that are translated into protein. However, recent genome‐wide studies have shown that the human genome is pervasively transcribed and produces many thousands of regulatory non‐protein‐coding RNAs (ncRNAs), including microRNAs, small interfering RNAs, PIWI‐interacting RNAs and various classes of long ncRNAs. It is now clear that these RNAs fulfil critical roles as transcriptional and post‐transcriptional regulators and as guides of chromatin‐modifying complexes. Here we review the biology of ncRNAs, focusing on the fundamental mechanisms by which ncRNAs facilitate normal development and physiology and, when dysfunctional, underpin disease. We also discuss evidence that intergenic regions associated with complex diseases express ncRNAs, as well as the potential use of ncRNAs as diagnostic markers and therapeutic targets. Taken together, these observations emphasize the need to move beyond the confines of protein‐coding genes and highlight the fact that continued investigation of ncRNA biogenesis and function will be necessary for a comprehensive understanding of human disease. Copyright © 2009 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Unravelling the genetic basis of renal diseases; from single gene to multifactorial disorders
Authors: McKnight Amy J , Currie Diane , Maxwell Alexander P 
Published: October 2009

AbstractChronic kidney disease is common with up to 5% of the adult population reported to have an estimated glomerular filtration rate of < 60 ml/min/1.73 m2. A large number of pathogenic mutations have been identified that are responsible for ‘single gene’ renal disorders, such as autosomal dominant polycystic kidney disease and X‐linked Alport syndrome. These single gene disorders account for < 15% of the burden of end‐stage renal disease that requires dialysis or kidney transplantation. It has proved more difficult to identify the genetic susceptibility underlying common, complex, multifactorial kidney conditions, such as diabetic nephropathy and hypertensive nephrosclerosis. This review describes success to date and explores strategies currently employed in defining the genetic basis for a number of renal disorders. The complementary use of linkage studies, candidate gene and genome‐wide association analyses are described and a collation of renal genetic resources highlighted. Copyright © 2009 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Ubiquitin as potential cerebrospinal fluid marker of Creutzfeldt–Jakob disease
Authors: Steinacker Petra , Rist Wolfgang , Swiatek‐de‐Lange Magdalena , Lehnert Stefan , Jesse Sarah , Pabst Alice , Tumani Hayrettin , von Arnim Christine A. F. , Mitrova Eva , Kretzschmar Hans A. , Lenter Martin , Wiltfang Jens , Otto Markus 
Published: October 2009

AbstractUntil today, a definite diagnosis of Creutzfeldt–Jakob disease (CJD) can only be made neuropathologically. At lifetime the early and differential diagnosis is often a problem. With SELDI we analyzed cerebrospinal fluid (CSF) from 32 CJD patients, 32 patients having other dementive diseases and 31 non‐demented control subjects for diagnosis‐dependent protein pattern differences. In a screening set of patients, peaks that discriminate best between groups were identified. These peaks were subsequently analyzed using an independent validation set of patients. Diagnostic accuracies were compared with established markers like tau protein and 14‐3‐3‐protein. Potential marker proteins were purified and identified by LC‐MS/MS. In the validation set only one peak of 8.6 kDa out of ten in the screening set could be confirmed. This protein was identified to be ubiquitin and increased levels in CSF (but not in serum) of CJD patients were confirmed by Western blot. Ubiquitin allows the correct diagnoses of that CJD cases missed by tau protein or 14‐3‐3‐protein. We conclude that ubiquitin is a promising additional CSF biomarker for diagnosis of CJD, especially in differential diagnostically difficult cases. The selective increase of ubiquitin in CSF of CJD patients might point to an involvement of ubiquitin in pathophysiological process.
Quantitative proteomic analysis of G‐protein signalling in Stagonospora nodorum using isobaric tags for relative and absolute quantification
Authors: Casey Tammy , Solomon Peter S. , Bringans Scott , Tan Kar‐Chun , Oliver Richard P. , Lipscombe Richard 
Published: October 2009

AbstractThe G protein α‐subunit (Gna1) in the wheat pathogen Stagonospora nodorum has previously been shown to be a critical controlling element in disease ontogeny. In this study, iTRAQ and 2‐D LC MALDI‐MS/MS have been used to characterise protein expression changes in the S. nodorum gna1 strain versus the SN15 wild‐type. A total of 1336 proteins were identified. The abundance of 49 proteins was significantly altered in the gna1 strain compared with the wild‐type. Gna1 was identified as having a significant regulatory role on primary metabolic pathways, particularly those concerned with NADPH synthesis or consumption. Mannitol dehydrogenase was up‐regulated in the gna1 strain while mannitol 1‐phosphate dehydrogenase was down‐regulated providing direct evidence of Gna1 regulation over this enigmatic pathway. Enzymatic analysis and growth assays confirmed this regulatory role. Several novel hypothetical proteins previously associated with stress and pathogen responses were identified as positively regulated by Gna1. A short‐chain dehydrogenase (Sch3) was also significantly less abundant in the gna1 strains. Sch3 was further characterised by gene disruption in S. nodorum by homologous recombination. Functional characterisation of the sch3 strains revealed their inability to sporulate in planta providing a further link to Gna1 signalling and asexual reproduction. These data add significantly to the identification of the regulatory targets of Gna1 signalling in S. nodorum and have demonstrated the utility of iTRAQ in dissecting signal transduction pathways.
Identification of complement C3f‐desArg and its derivative for acute leukemia diagnosis and minimal residual disease assessment
Authors: Liang Tingting , Wang Na , Li Wei , Li Ailing , Wang Jie , Cui Jiuwei , Liu Nian , Li Yan , Li Ling , Yang Guozi , Du ZhongHua , Li Dan , He Kun , Wang Guanjun 
Published: October 2009

AbstractTherapeutic conditions for acute leukemia (AL) mainly rely on diagnosis and detection of minimal residual disease (MRD). However, no serum biomarker has been available for clinicians to make diagnosis of AL and assessment of MRD. In this study, we performed bead fractionation/MALDI‐TOF‐MS analysis on sera from patients with AL. Support vector machine algorithm was used to obtain diagnostic model that discriminated proteomic spectra of patients with AL from that of controls. Twenty‐six features with p<0.00001 had optimal discriminatory performance, with 97% sensitivity and 100% specificity. Statistical analysis revealed that two peptides with m/z 1778 and 1865 were gradually decreased in their relative intensities with increase of remission degree. Moreover, the peptide with m/z 1865 was also found to be correlated with AL types. With FT‐ICR‐MS detection, both the peptides were identified as fragments of complement C3f. Linear regression analysis showed that the combined use of them could discriminate PML/RARα positive M3 from molecular remission M3. Two fragments of complement C3f were significantly correlated with MRD levels and could be used for clinical practice in MRD assessment.
Helix propensities calculations for amino acids in alanine based peptides using Jarzynski's equality
Authors: Echeverria Ignacia , Amzel L. Mario 
Published: October 2009

AbstractJarzynski's equality (Jarzynski, Phys Rev E 1997; 56:5018 and Jarzynski, Phys Rev Lett 1997; 78:2690) relates equilibrium free energy differences between two states A and B to the work done when the system is driven repeatedly and irreversibly from an equilibrium state A to equilibrium state B. We present calculations of helix propensities using a novel procedure based on this equality. In particular, a work probability distribution is built based on combinations of multi‐step trajectories that give representative work distributions without requiring computing an unreasonable large number of trajectories between states. A small number of trajectories (15) are used to construct a distribution that contains millions of work values. This distribution is used to calculate ΔGAB using Jarsynski's equation. To apply and test this method, we used as a model system a dodeca‐alanine helix, analyzing its extension using mechanical force. This helix was used as the basis of a host guest system in which two of the 12 residues are substituted by some other amino acid (as the guests). The differences between the unfolding free energies of the substituted peptides and the all‐alanine peptide provided values for ΔΔG that can be interpreted as the helix propensities of each amino acid. Results show good correlation with the experimental measurements of Baldwin and coworkers (Chakrabartty et al., Protein Sci 1994; 3:843–852). Proteins 2010. © 2009 Wiley‐Liss, Inc.
Looking across industries to improve human reliability data for quantitative risk analyses
Authors: Schneider Roxy 
Published: October 2009

AbstractAlthough meticulous work is often done to determine failure probabilities of Safety Instrumented Systems for Quantitative Risk Assessments (QRAs), the work done to determine failure probabilities of humans performing specific tasks are often less rigorously performed. Yet, human reliability data is just as important to the quality and accuracy of the QRA as non‐human failure probabilities. The problem lies in the lack of availability of statistically reliable human failure probabilities for various tasks within the process industries. This article will present and analyze the strengths and weaknesses of a variety of sources of such data. The various methods of data collection and analysis will be discussed and as will the quality and applications of each methods' data output. More specifically, emphasis will be placed on discussion of the US Nuclear Regulatory Commission's Human Event Repository and Analysis program and the possible applicability and worth of the program to the process industries. This presentation is based on work done for the Center for Chemical Process Safety's Human Reliability Project. © 2009 American Institute of Chemical Engineers Process Saf Prog, 2010
The effect of TiO2 on the microstructural and electrical properties of low voltage varistor based on (Sn,Ti)O2 ceramics
Authors: Leite D. R. , Cilense M. , Orlandi M. O. , Bueno P. R. , Longo E. , Varela J. A. 
Published: October 2009

AbstractSnO2·CoO·Nb2O5 ceramics doped with TiO2 have been prepared by a conventional oxide method which focuses its application on low voltage varistors. The dopant was added in 0.5 and 1.0% molar concentrations, and the samples were investigated by X‐ray diffraction, scanning electron microscopy, current vs. voltage, and impedance measurements. The electron microscopy results showed an increase in the mean grain size of ceramics with the addition of TiO2, which is related to the effect of the dopant on the matrix. The electrical characterization showed that the addition of TiO2 in 1 mol% provides a system with a good nonlinear coefficient (8.7) and a breakdown electrical field of 617 V/cm. These results indicate that this composition can be applied as a low voltage varistor. The impedance data showed that the voltage barrier at the grain boundary is a back‐to‐back Schottky‐type, and it was demonstrated that the addition of TiO2 does not significantly modify the barrier.
Identifying the electron spin resonance of conduction electrons in alkali doped SWCNTs
Authors: Galambos M. , Fábián G. , Simon F. , Ćirić L. , Forró L. , Korecz L. , Rockenbauer A. , Koltai J. , Zólyomi V. , Rusznyák Á. , Kürti J. , Nemes N. M. , Dóra B. , Peterlik H. , Pfeiffer R. , Kuzmany H. , Pichler T. 
Published: October 2009

AbstractWe study the electron spin resonance (ESR) signal of pristine and potassium doped SWCNTs. We identify signals of a super‐paramagnetic background, a low intensity impurity, and of the conduction electron spin resonance (CESR). The latter only appears upon the alkali atom doping. To identify the CESR signal, we critically assess whether it could come from residual graphitic carbon, which we clearly exclude. We give accurate values for the signal intensities and the corresponding concentration of spins and for the g‐factors. The CESR signal intensity allows to determine the density of states on the SWCNT assembly.
Estimation of prediction error for survival models
Authors: Lawless Jerald F. , Yuan Yan 
Published: October 2009

AbstractWhen statistical models are used to predict the values of unobserved random variables, loss functions are often used to quantify the accuracy of a prediction. The expected loss over some specified set of occasions is called the prediction error. This paper considers the estimation of prediction error when regression models are used to predict survival times and discusses the use of these estimates. Extending the previous work, we consider both point and confidence interval estimations of prediction error, and allow for variable selection and model misspecification. Different estimators are compared in a simulation study for an absolute relative error loss function, and results indicate that cross‐validation procedures typically produce reliable point estimates and confidence intervals, whereas model‐based estimates are sensitive to model misspecification. Links between performance measures for point predictors and for predictive distributions of survival times are also discussed. The methodology is illustrated in a medical setting involving survival after treatment for disease. Copyright © 2009 John Wiley & Sons, Ltd.
Cross‐profiling for Java processors
Authors: Binder Walter , Schoeberl Martin , Moret Philippe , Villazón Alex 
Published: October 2009

AbstractPerformance evaluation of embedded software is essential in an early development phase so as to ensure that the software will run on the embedded device's limited computing resources. The prevailing approaches either require the deployment of the software on the embedded target, which can be tedious and may be impossible in an early development phase, or rely on simulation, which can be very slow. In this article, we introduce a customizable cross‐profiling framework for embedded Java processors, including processors featuring a method cache. The developer profiles the embedded software in the host environment, completely decoupled from the target system, on any standard Java virtual machine, but the generated profiles represent the execution time metric of the target system. Our cross‐profiling framework is based on bytecode instrumentation. We identify several pointcuts in the execution of bytecode that need to be instrumented in order to estimate the CPU cycle consumption on the target system. An evaluation using the JOP embedded Java processor as target confirms that our approach reconciles high profile accuracy with moderate overhead. Our cross‐profiling framework also enables the performance evaluation of new processor architectures before they are implemented. As a case study, we explore the performance impact of various processor design choices and optimizations, such as different cache sizes or pipeline organizations, and come up with an improved processor design that yields speedups of up to 40% on standard Java benchmarks. Copyright © 2009 John Wiley & Sons, Ltd.
The Egf Receptor-Sox2-Egf Receptor Feedback Loop Positively Regulates the Self-Renewal of Neural Precursor Cells
Authors: Hu Qikuan , Zhang Lirong , Wen Jinhua , Wang Shuling , Li Meiyu , Feng Ruopeng , Yang Xiaolong , Li Lingsong 
Published: October 2009

Abstract
The transcriptional factor Sox2 and epidermal growth factor receptor (Egfr)-mediated signaling are both required for self-renewal of neural precursor cells (NPCs). However, the mechanism by which these factors coordinately regulate this process is largely unknown. Here we show that Egfr-mediated signaling promotes Sox2 expression, which in turn binds to the Egfr promoter and directly upregulates Egfr expression. Knockdown of Sox2 by RNA interference downregulates Egfr expression and attenuates colony formation of NPCs, whereas overexpression of Sox2 elevates Egfr expression and promotes NPC self-renewal. Moreover, the effect of Sox2 on NPC self-renewal is completely inhibited by AG1478, a specific inhibitor for Egfr; it is also inhibited by LY294002 and U0126, selective antagonists for phosphatidylinositol 3-kinase (PI3K) and extracellular signal-regulated kinase (Erk1/2), respectively. Collectively, we conclude that NPC self-renewal is enhanced through a novel cellular feedback loop with mutual regulation of Egfr and Sox2.
Evaluating the effect of masonry infills on natural period of buildings with moment‐resisting frame
Authors: Barghi Mostafa , Azadbakht Mohsen 
Published: October 2009

AbstractBuilding design codes generally provide empirical formulas for estimating the fundamental period. These formulas are developed on the basis of observed periods of real buildings during ground motion, and the period is generally expressed as a function of building height, type (frame or shear wall), etc. In this study, the fundamental period of vibration of a series of regular steel‐framed buildings is studied using finite element modelling and modal eigenvalue analysis including the effect of infill. It has been found that when the models do not include infill, the period given by the analysis is significantly longer than that predicted by the codes equation. However, when the effect of infill is included in the models, the time periods determined from eigenvalue analysis were remarkably close to those predicted by the code formulas. The finding of the study has shown us a practical way to determine the fundamental period of steel frames using rational approaches like modal analysis. After an evaluation of the results, a modified formula has been proposed for estimation of natural period of steel buildings with moment‐resisting frame system having infill. Using the proposed formula, we can estimate natural period of this kind of structural systems more accurately than the one approximated by code formula. Copyright © 2009 John Wiley & Sons, Ltd.