bluuebunny/crossref_metadata_2025_split huggingface dataset:
CALCB splice region pathogenic variants leading to plasma cell neurotropic enrichment in type 1 autoimmune pancreatitis
Authors: Liu Qi-cai , Chen Falin , Wu Chao-yang , Gao Feng , Zhuang Ze-hao , Chen Jin-tong , Cai Bin , Zhang Tianming , Guo Ling , Lin Li-qing , Zhao Cheng-fei , Lin Xin-hua 
Published: February 2017

AbstractRecently, we have demonstrated that PRSS1 mutations cause ectopic trypsinogen activation and thereby result in type 1 autoimmune pancreatitis (AIP). However, the molecules involved in inducing obliterative vasculitis and perineural inflammation in the pancreas are not well-described. The present study applied whole-exome sequencing (WES) to determine the underlying etiology and revealed novel missense splice region variants, CALCB c.88T>C (p.Ser30Pro) and IR [1]-mutants, in 2 of the 3 families and 2 of 26 unrelated patients with type 1 AIP. In vitro, both of the mutants displayed decreased βCGRP, ERK1/2 phosphorylation, and co-localized with endoplasmic reticulum and Golgi apparatus. The novel pathogenic variant identified in this case should contribute to our understanding of the expanding spectrum of AIP.
A two-channel, spectrally degenerate polarization entangled source on chip
Authors: Sansoni Linda , Luo Kai Hong , Eigner Christof , Ricken Raimund , Quiring Viktor , Herrmann Harald , Silberhorn Christine 
Published: February 2017

AbstractIntegrated optics provides the platform for the experimental implementation of highly complex and compact circuits for quantum information applications. In this context integrated waveguide sources represent a powerful resource for the generation of quantum states of light due to their high brightness and stability. However, the confinement of the light in a single spatial mode limits the realization of multi-channel sources. Due to this challenge one of the most adopted sources in quantum information processes, i.e. a source which generates spectrally indistinguishable polarization entangled photons in two different spatial modes, has not yet been realized in a fully integrated platform. Here we overcome this limitation by suitably engineering two periodically poled waveguides and an integrated polarization splitter in lithium niobate. This source produces polarization entangled states with fidelity of $${\mathcal F} \mathrm{=0.973}\pm 0.003$$
F
= 0.973
±
0.003
and a test of Bell’s inequality results in a violation larger than 14 standard deviations. It can work both in pulsed and continuous wave regime. This device represents a new step toward the implementation of fully integrated circuits for quantum information applications.
The E3 ligase Mule protects the heart against oxidative stress and mitochondrial dysfunction through Myc-dependent inactivation of Pgc-1α and Pink1
Authors: Dadson Keith , Hauck Ludger , Hao Zhenyue , Grothe Daniela , Rao Vivek , Mak Tak W. , Billia Filio 
Published: February 2017

AbstractCardiac homeostasis requires proper control of protein turnover. Protein degradation is principally controlled by the Ubiquitin-Proteasome System. Mule is an E3 ubiquitin ligase that regulates cellular growth, DNA repair and apoptosis to maintain normal tissue architecture. However, Mule’s function in the heart has yet to be described. In a screen, we found reduced Mule expression in left ventricular samples from end-stage heart failure patients. Consequently, we generated conditional cardiac-specific Mule knockout (Mule fl/fl(y);mcm) mice. Mule ablation in adult Mule fl/fl(y);mcm mice prevented myocardial c-Myc polyubiquitination, leading to c-Myc accumulation and subsequent reduced expression of Pgc-1α, Pink1, and mitochondrial complex proteins. Furthermore, these mice developed spontaneous cardiac hypertrophy, left ventricular dysfunction, and early mortality. Co-deletion of Mule and c-Myc rescued this phenotype. Our data supports an indispensable role for Mule in cardiac homeostasis through the regulation of mitochondrial function via maintenance of Pgc-1α and Pink1 expression and persistent negative regulation of c-Myc.
The impact of variable commitment in the Naming Game on consensus formation
Authors: Niu Xiang , Doyle Casey , Korniss Gyorgy , Szymanski Boleslaw K. 
Published: February 2017

AbstractThe Naming Game has proven to be an important model of opinion dynamics in complex networks. It is significantly enriched by the introduction of nodes committed to a single opinion. The resulting model is still simple but captures core concepts of opinion dynamics in networks. This model limitation is rigid commitment which never changes. Here we study the effect that making commitment variable has on the dynamics of the system. Committed nodes are assigned a commitment strength, w, defining their willingness to lose (in waning), gain (for increasing) or both (in variable) commitment to an opinion. Such model has committed nodes that can stick to a single opinion for some time without losing their flexibility to change it in the long run. The traditional Naming Game corresponds to setting w at infinity. A change in commitment strength impacts the critical fraction of population necessary for a minority consensus. Increasing w lowers critical fraction for waning commitment but increases this fraction for increasing commitment. Further, we show that if different nodes have different values of w, higher standard deviation of w increases the critical fraction for waning commitment and decrease this fraction for increasing commitment.
Engineering an endocrine Neo-Pancreas by repopulation of a decellularized rat pancreas with islets of Langerhans
Authors: Napierala H. , Hillebrandt K.-H. , Haep N. , Tang P. , Tintemann M. , Gassner J. , Noesser M. , Everwien H. , Seiffert N. , Kluge M. , Teegen E. , Polenz D. , Lippert S. , Geisel D. , Reutzel Selke A. , Raschzok N. , Andreou A. , Pratschke J. , Sauer I. M. , Struecker B. 
Published: February 2017

AbstractDecellularization of pancreata and repopulation of these non-immunogenic matrices with islets and endothelial cells could provide transplantable, endocrine Neo- Pancreata. In this study, rat pancreata were perfusion decellularized and repopulated with intact islets, comparing three perfusion routes (Artery, Portal Vein, Pancreatic Duct). Decellularization effectively removed all cellular components but conserved the pancreas specific extracellular matrix. Digital subtraction angiography of the matrices showed a conserved integrity of the decellularized vascular system but a contrast emersion into the parenchyma via the decellularized pancreatic duct. Islets infused via the pancreatic duct leaked from the ductular system into the peri-ductular decellularized space despite their magnitude. TUNEL staining and Glucose stimulated insulin secretion revealed that islets were viable and functional after the process. We present the first available protocol for perfusion decellularization of rat pancreata via three different perfusion routes. Furthermore, we provide first proof-of-concept for the repopulation of the decellularized rat pancreata with functional islets of Langerhans. The presented technique can serve as a bioengineering platform to generate implantable and functional endocrine Neo-Pancreata.
Gestational Weight Gain and Fetal-Maternal Adiponectin, Leptin, and CRP: results of two birth cohorts studies
Authors: Logan Chad A. , Bornemann Rebecca , Koenig Wolfgang , Reister Frank , Walter Viola , Fantuzzi Giamila , Weyermann Maria , Brenner Hermann , Genuneit Jon , Rothenbacher Dietrich 
Published: February 2017

AbstractGestational weight gain (GWG) is an important modifiable factor known to influence fetal outcomes including birth weight and adiposity. Unlike behaviors such as smoking and alcohol consumption, the effect of GWG throughout pregnancy on fetal development and other outcomes has not been extensively studied. The aim of this study was to investigate the relationship of GWG with endocrine factors such as adiponectin, leptin, and C-reactive protein which may be associated with inflammatory response, fetal growth, and adiposity later in life. Data were obtained from the Ulm Birth Cohort Study (UBCS) and the Ulm SPATZ Health Study, two methodologically similar birth cohort studies including newborns and their mothers recruited from 11/2000–11/2001 and 04/2012–05/2013. In the two included birth cohorts we consistently observed statistically significant positive associations between GWG beginning as early as the second trimester with fetal cord blood leptin and stronger association beginning as early as the first trimester with post-delivery maternal serum leptin. Total weight gain exceeding commonly accepted recommended guidelines was consistently associated with higher leptin levels in both cord blood and post-delivery maternal serum. These results suggest a potential pathomechanistic link between fetal environment and surrogate markers of long-term health.
Methanol-Independent Protein Expression by AOX1 Promoter with trans-Acting Elements Engineering and Glucose-Glycerol-Shift Induction in Pichia pastoris
Authors: Wang Jinjia , Wang Xiaolong , Shi Lei , Qi Fei , Zhang Ping , Zhang Yuanxing , Zhou Xiangshan , Song Zhiwei , Cai Menghao 
Published: February 2017

AbstractThealcohol oxidase 1promoter (PAOX1) ofPichia pastorisis commonly used for high level expression of recombinant proteins. While the safety risk of methanol and tough process control for methanol induction usually cause problems especially in large-scale fermentation. By testing the functions of trans-actingelements of PAOX1and combinatorially engineering of them, we successfully constructed a methanol-free PAOX1start-up strain, in which, three transcription repressors were identified and deleted and, one transcription activator were overexpressed. The strain expressed 77% GFP levels in glycerol compared to the wide-type in methanol. Then, insulin precursor (IP) was expressed, taking which as a model, we developed a novel glucose-glycerol-shift induced PAOX1start-up for this methanol-free strain. A batch phase with glucose of 40 g/L followed by controlling residual glucose not lower than 20 g/L was compatible for supporting cell growth and suppressing PAOX1. Then, glycerol induction was started after glucose used up. Accordingly, an optimal bioprocess was further determined, generating a high IP production of 2.46 g/L in a 5-L bioreactor with dramatical decrease of oxygen consumption and heat evolution comparing with the wild-type in methanol. This mutant and bioprocess represent a safe and efficient alternative to the traditional glycerol-repressed/methanol-induced PAOX1system.
A Comparative Study of the Precision of Carstens and Northern Digital Instruments Electromagnetic Articulographs
Authors: Savariaux Christophe , Badin Pierre , Samson Adeline , Gerber Silvain 
Published: February 2017

Purpose
This study compares the precision of the electromagnetic articulographs used in speech research: Northern Digital Instruments' Wave and Carstens' AG200, AG500, and AG501 systems.

Method
The fluctuation of distances between 3 pairs of sensors attached to a manually rotated device that can position them inside the measurement volumes was determined. For each device, 2 precision estimates made on the basis of the 95% quantile range of these distances (QR95) were defined: The local QR95 was computed for bins around specific rotation angles, and the global QR95 was computed for all angles pooled.

Results
For all devices, although the local precision lies around 0.1 cm, the global precision is much more worrisome, ranging from 0.03 cm to 2.18 cm, and displays large variations as a function of the position of the sensors in the measurement volume. No influence of the rotational speed was found. The AG501 produced—by far—the lowest errors, in particular concerning the global precision.

Conclusions
The local precision can be considered suitable for speech articulatory measurements, but the variations of the global precision need to be taken into account by the knowledge of the spatial distribution of errors. A guideline for good practice in EMA recording is proposed for each system.
Incidence of Secondary Hydrocephalus after Excision of Huge Encephaloceles in Neonates: Case Study
Authors: Refaat Mohamed , Reda Mohamed , Refaee Ehab 
Published: February 2017

Background Encephaloceles presents as a protrusion of the cranial contents through a defect in the cranium. The most common sites of occurrence are the occipital and frontonasal regions. The surgical outcome is reported to be satisfactory; however, the incidence of hydrocephalus in patients with encephaloceles is variable in the literature. This study investigated the relationship between the size of the encephaloceles and the occurrence of hydrocephalus.
Patient and Methods Data of all neonates with encephaloceles who presented to our institution from September 2012 to September 2014 were collected. Surgery was performed during the first 2 weeks of age. Encephaloceles with a maximal diameter > 10 cm were included in the study, and the clinical picture, surgical technique, pre- and postoperative imaging, and follow-up were analyzed.
Results Nineteen cases were included in this study. The mean follow-up period was 7 months. Sixteen cases were occipital; three were frontal. In all patients the maximum diameter was > 10 cm. However, in four patients it was > 18 cm. Postoperative ventriculomegaly occurred in seven cases. Of these, four patients needed a permanent ventriculoperitoneal shunt implantation (21%). Wound dehiscence occurred in two patients who required secondary sutures with a favorable outcome. One patient died 2 weeks after the surgery due to a poor general condition and wound infection.
Conclusion Early surgical excision provides effective treatment of huge encephaloceles. Overall, 21% of cases require cerebrospinal fluid (CSF) diversion afterward depending on associated anomalies. Despite their size, giant encephaloceles can have an excellent prognosis with no need for further treatment or CSF diversion.
Acoustic backscatter by schools of adult Atlantic mackerel
Authors: Gorska Natalia , Korneliussen Rolf J. , Ona Egil 
Published: February 2017

Abstract
Gorska, N., Korneliussen, R. J., and Ona, E. 2007. Acoustic backscatter by schools of adult Atlantic mackerel. – ICES Journal of Marine Science, 64: 1145–1151. The extent of acoustic backscatter by schools of adult Atlantic mackerel (Scomber scombrus) is investigated to improve biomass estimates. Previous studies involving modelled scattering from individual mackerel showed that backscattering at high frequencies is dominated by the contribution from the backbone. Accurate predictions of the scattering spectra require consideration of backscattering from the entire skeleton, including details of the bone shapes and their acoustic properties. Here, the backscattering cross-sections from mackerel flesh and backbone are estimated theoretically from 18 to 364 kHz and averaged over fish size and tilt angle, then compared with in situ measurements of volume backscattering from mackerel schools. Based on the comparisons, some gross features of the observed relative frequency response are explained, and recommendations for further studies suggested.
222Rn in the Antarctic Peninsula During 1986
Authors: Pereira E.B. , Setzer A.W. , Cavalcanti I.F.A. 
Published: February 2017

Abstract
222Rn was continuously measured at the Brazilian Antarctic Station (62 oS, 58 oW) during the year of 1986. Baseline radon concentration averaged 0.02 Bq.m-3 with surges peaking at 0.4 Bq.m-3. The data exhibited a characteristic periodicity of about 25 days and a strong positive association with short term fluctuations of atmospheric temperature. No seasonal variation of radon were observed. Interpretation of the radon surges with reference to synoptic charts and weather satellite pictures showed that the continental influence of radon at the Antarctic Peninsula is very small and comes only from the tip of the South American cone.
Windows 7 Antiforensics: A Review and a Novel Approach
Authors: Eterovic‐Soric Brett , Choo Kim‐Kwang Raymond , Mubarak Sameera , Ashman Helen 
Published: February 2017

AbstractIn this paper, we review literature on antiforensics published between 2010 and 2016 and reveal the surprising lack of up‐to‐date research on this topic. This research aims to contribute to this knowledge gap by investigating different antiforensic techniques for devices running Windows 7, one of the most popular operating systems. An approach which allows for removal or obfuscation of most forensic evidence is then presented. Using the Trojan software DarkComet RAT as a case study, we demonstrate the utility of our approach and that a Trojan Horse infection may be a legitimate possibility, even if there is no evidence of an infection on a seized computer's hard drive. Up‐to‐date information regarding how forensic artifacts can be compromised will allow relevant stakeholders to make informed decisions when deciding the outcome of legal cases involving digital evidence.
Letter: should direct‐acting antiviral drugs be used concomitantly with chemotherapeutic drugs in hepatitis C virus‐infected patients with cancer?
Authors: Jiang J.‐F. , Sun J. , Shi J. , Liu X. 
Published: February 2017

Linked ContentThis article is linked to Economides et al papers. To view these articles visit https://doi.org/10.1111/apt.13825 and https://doi.org/10.1111/apt.13937.
In search of making a difference: Mexico in the OECD international development co‐operation architecture
Authors: Villanueva Ulfgard Rebecka , López Lorena 
Published: February 2017

AbstractA major challenge for Middle‐Income Countries is to ‘make a difference’ in global issues. The article examines Mexico's compromise in this regard within the OECD international development co‐operation architecture. It highlights some of the footprints of Mexican diplomacy since the OECD membership (1994), through the Working Party on Aid Effectiveness, up to the launch of the Global Partnership for Effective Development Co‐operation (GPEDC), demonstrating how Mexico gradually increased its profile and responsibilities in specific international negotiation frameworks where its dual nature as donor/recipient is precisely contributing to ‘making a difference’. Suggesting that Mexico could expand its South‐South Co‐operation (SSC) even further, the authors argue that Mexico's quest is constrained by a certain consensus on the predominant development paradigm manifested in the GPEDC.
Modelling and management options for salmonid sport fisheries: A case study from Patagonia, Argentina
Authors: Rechencq M. , Vigliano P. H. , Lippolt G. E. , Alonso M. F. , Macchi P. J. , Alvear P. A. , Hougham V. , Denegri M. A. , Blasetti G. , Juncos R. , Fernandez M. V. , Lallement M. E. , Juarez S. M. 
Published: February 2017

AbstractSalmonid sport fishery management in Argentinian Patagonia is usually guided by stakeholder perceptions, which do not consider the biological and ecological constraints acting upon aquatic resources. An example of this are management policies for Traful Lake, where Atlantic salmon, Salmo salar L., was actively stocked during the last decade in an attempt to change the fish assemblage structure and to create a highly valuable sport fishery. This study assesses the likelihood of these policies achieving such assemblage structure changes through the study of affluent‐stream suitability as spawning and breeding grounds, lake fish assemblage parameters, trophic diversity and possible competition due to diet overlap. This study also assesses alternative management practices through population simulations and bioenergetic modelling under diverse catch‐size limit scenarios. This study concludes that it is not advisable to try to generate a unique and distinctive sport fishery by stocking Atlantic salmon in an already renowned fishery in Northern Patagonia. Instead, a more comprehensive framework for decision‐making is suggested, involving short‐term studies that go beyond the specific problem of the target species and incorporate the whole fish assemblage using diverse approaches and modelling strategies.
Nocardia liver abscess post liver transplantation—A rare presentation
Authors: Hanchanale Pavan , Jain Mayank , Varghese Joy , V Jayanthi , Rela Mohamed 
Published: February 2017

AbstractNocardiosis is usually a disseminated disease seen in immunocompromised individuals. We herein present a rare case of isolated Nocardia liver abscess post liver transplantation. The patient responded well to treatment and is on long‐term antibiotics for Nocardia infection.
Do Women With Atrial Fibrillation Experience More Severe Strokes?
Authors: Lang Clemens , Seyfang Leonhard , Ferrari Julia , Gattringer Thomas , Greisenegger Stefan , Willeit Karin , Toell Thomas , Krebs Stefan , Brainin Michael , Kiechl Stefan , Willeit Johann , Lang Wilfried , Knoflach Michael 
Published: February 2017

Background and Purpose—
Ischemic strokes associated with atrial fibrillation (AF) are more severe than those of other cause. We aim to study potential sex effects in this context.

Methods—
In this cross-sectional study, 74 425 adults with acute ischemic stroke from the Austrian Stroke Unit Registry were included between March 2003 and January 2016. In 63 563 patients, data on the National Institutes of Health Stroke Scale on admission to the stroke unit, presence of AF, vascular risk factors, and comorbidities were complete. Analysis was done by a multivariate regression model.

Results—

Stroke severity in general increased with age. AF-related strokes were more severe than strokes of other causes. Sex-related differences in stroke severity were only seen in stroke patients with AF. Median (Q
25
,
75
) National Institutes of Health Stroke Scale score points were 9 (4,17) in women and 6 (3,13) in men (
P
<0.001). The interaction between AF and sex on stroke severity was independent of age, previous functional status, vascular risk factors, and vascular comorbidities and remained significant in various subgroups.

Conclusions—
Women with AF do not only have an increased risk of stroke when compared with men but also experience more severe strokes.
Costal cartilage transplantation for treatment of growth plate injury in a rabbit model
Authors: Otsuki D. , Yoshida K. , Kobayashi M. , Hamano D. , Higuchi C. , Yoshikawa H. 
Published: February 2017

Objectives The purpose of this study is to evaluate the effects of allogenic costal cartilage transplantation on preventing bony bridge formation and angular deformities for the treatment of partial growth plate injury using a rabbit model. Methods An experimental model of partial growth injury was created by resecting the medial part of the proximal tibial growth plate in male six-week-old New Zealand White rabbits. The rabbits were divided into four groups: no surgery; no transplantation; bone wax transplantation; and allogenic costal cartilage transplantation. The angular deformities of the tibia and bony bridge were analysed using radiographs and microcomputed tomography, and the repair of the injured growth plate cartilage and bony bridge formation rate were histologically evaluated. Results On radiographic evaluation, the varus deformities in the costal cartilage group were significantly improved compared with the no transplantation group at four and eight weeks after operation and with the bone wax group at eight weeks after operation. Micro-computed tomography showed that the bony bridge formation was prevented in the bone wax and costal cartilage groups. Histological findings showed that the bony bridge formation in the bone wax and costal cartilage group was decreased. In addition, the growth plate was continuous and stained with safranin O and immunohistochemically stained for type II collagen. Conclusion Transplantation of costal cartilage improved angular deformities and decreased bony bridge formation in the partial growth plate injury. Costal cartilage might be a suitable graft for the treatment of growth plate injury.