bluuebunny/crossref_metadata_2025_split huggingface dataset:
Type 0 Spinal Muscular Atrophy: Further Delineation of Prenatal and Postnatal Features in 16 Patients
Authors: Grotto Sarah , Cuisset Jean-Marie , Marret Stéphane , Drunat Séverine , Faure Patricia , Audebert-Bellanger Séverine , Desguerre Isabelle , Flurin Vincent , Grebille Anne-Gaëlle , Guerrot Anne-Marie , Journel Hubert , Morin Gilles , Plessis Ghislaine , Renolleau Sylvain , Roume Joëlle , Simon-Bouy Brigitte , Touraine Renaud , Willems Marjolaine , Frébourg Thierry , Verspyck Eric , Saugier-Veber Pascale 
Published: September 2016

Background: Spinal muscular atrophy (SMA) is caused by homozygous inactivation of the SMN1 gene. The SMN2 copy number modulates the severity of SMA. The 0 SMN1/1 SMN2 genotype, the most severe genotype compatible with life, is expected to be associated with the most severe form of the disease, called type 0 SMA, defined by prenatal onset. Objective: The aim of the study was to review clinical features and prenatal manifestations in this rare SMA subtype. Methods: SMA patients with the 0 SMN1/1 SMN2 genotype were retrospectively collected using the UMD-SMN1 France database. Results: Data from 16 patients were reviewed. These 16 patients displayed type 0 SMA. At birth, a vast majority had profound hypotonia, severe muscle weakness, severe respiratory distress, and cranial nerves involvement (inability to suck/swallow, facial muscles weakness). They showed characteristics of fetal akinesia deformation sequence and congenital heart defects. Recurrent episodes of bradycardia were observed. Death occurred within the first month. At prenatal stage, decreased fetal movements were frequently reported, mostly only by mothers, in late stages of pregnancy; increased nuchal translucency was reported in about half of the cases; congenital heart defects, abnormal amniotic fluid volume, or joint contractures were occasionally reported. Conclusion: Despite a prenatal onset attested by severity at birth and signs of fetal akinesia deformation sequence, prenatal manifestations of type 0 SMA are not specific and not constant. As illustrated by the frequent association with congenital heart defects, type 0 SMA physiopathology is not restricted to motor neuron, highlighting that SMN function is critical for organogenesis.
Dietary olive oil induces cannabinoid CB2 receptor expression in adipose tissue of ApcMin/+ transgenic mice
Authors: Notarnicola Maria , Tutino Valeria , Tafaro Angela , Bianco Giusy , Guglielmi Emilia , Caruso Maria Gabriella 
Published: September 2016

BACKGROUND: Cannabinoid- 2 (CB2) receptor is known for its anti-obesity effects silencing the activated immune cells that are key drivers of metabolic syndrome and inflammation. Nutritional interventions in experimental models of carcinogenesis have been demonstrated to modulate tissue inflammation state and proliferation. OBJECTIVE: Aim of this study was to test, in ApcMin/+ mice, whether a diet enriched with olive oil, omega- 3 and omega-6- PUFAs affects the adipose tissue inflammation status. METHODS: Four groups of animal were studied: ST group, receiving a standard diet; OO group, receiving the standard diet in which soybean oil (source of fats) was replaced with olive oil; OM-3 group, receiving the standard diet in which soybean oil was replaced with salmon oil; OM-6 group, receiving the standard diet in which soybean oil was replaced with oenothera oil. Gene and protein expression, in adipose tissue, were evaluated by RT-PCR and Western Blotting, respectively. Enzymatic activities were assayed by fluorescent and radiometric method, where appropriated. RESULTS: The diet enriched with olive oil significantly induced CB2 receptor expression and it was able to control inflammatory and proliferative activity of mice adipose tissue. CONCLUSIONS: The present findings open opportunities for developing novel nutritional strategies considering olive oil a key ingredient of a healthy dietary pattern.
INFERENCE OF HEATING PROPERTIES FROM “HOT” NON-FLARING PLASMAS IN ACTIVE REGION CORES. I. SINGLE NANOFLARES
Authors: Barnes W. T. , Cargill P. J. , Bradshaw S. J. 
Published: September 2016

ABSTRACT
The properties that are expected of “hot” non-flaring plasmas due to nanoflare heating in active regions are investigated using hydrodynamic modeling tools, including a two-fluid development of the Enthalpy Based Thermal Evolution of Loops code. Here we study a single nanoflare and show that while simple models predict an emission measure distribution extending well above 10 MK, which is consistent with cooling by thermal conduction, many other effects are likely to limit the existence and detectability of such plasmas. These include: differential heating between electrons and ions, ionization non-equilibrium, and for short nanoflares, the time taken for the coronal density to increase. The most useful temperature range to look for this plasma, often called the “smoking gun” of nanoflare heating, lies between 106.6 and 107 K. Signatures of the actual heating may be detectable in some instances.
LITHIUM DEPLETION IS A STRONG TEST OF CORE-ENVELOPE RECOUPLING
Authors: Somers Garrett , Pinsonneault Marc H. 
Published: September 2016

ABSTRACT
Rotational mixing is a prime candidate for explaining the gradual depletion of lithium from the photospheres of cool stars during the main sequence. However, previous mixing calculations have relied primarily on treatments of angular momentum transport in stellar interiors incompatible with solar and stellar data in the sense that they overestimate the internal differential rotation. Instead, recent studies suggest that stars are strongly differentially rotating at young ages but approach a solid body rotation during their lifetimes. We modify our rotating stellar evolution code to include an additional source of angular momentum transport, a necessary ingredient for explaining the open cluster rotation pattern, and examine the consequences for mixing. We confirm that core-envelope recoupling with a ∼20 Myr timescale is required to explain the evolution of the mean rotation pattern along the main sequence, and demonstrate that it also provides a more accurate description of the Li depletion pattern seen in open clusters. Recoupling produces a characteristic pattern of efficient mixing at early ages and little mixing at late ages, thus predicting a flattening of Li depletion at a few Gyr, in agreement with the observed late-time evolution. Using Li abundances we argue that the timescale for core-envelope recoupling during the main sequence decreases sharply with increasing mass. We discuss the implications of this finding for stellar physics, including the viability of gravity waves and magnetic fields as agents of angular momentum transport. We also raise the possibility of intrinsic differences in initial conditions in star clusters using M67 as an example.
TRANSITION REGION AND CHROMOSPHERIC SIGNATURES OF IMPULSIVE HEATING EVENTS. I. OBSERVATIONS
Authors: Warren Harry P. , Reep Jeffrey W. , Crump Nicholas A. , Simões Paulo J. A. 
Published: September 2016

ABSTRACT
We exploit the high spatial resolution and high cadence of the Interface Region Imaging Spectrograph (IRIS) to investigate the response of the transition region and chromosphere to energy deposition during a small flare. Simultaneous observations from the Reuven Ramaty High Energy Solar Spectroscopic Imager provide constraints on the energetic electrons precipitating into the flare footpoints, while observations of the X-Ray Telescope, Atmospheric Imaging Assembly, and Extreme Ultraviolet Imaging Spectrometer (EIS) allow us to measure the temperatures and emission measures from the resulting flare loops. We find clear evidence for heating over an extended period on the spatial scale of a single IRIS pixel. During the impulsive phase of this event, the intensities in each pixel for the Si iv 1402.770 Å, C ii 1334.535 Å, Mg ii 2796.354 Å, and O i 1355.598 Å emission lines are characterized by numerous small-scale bursts typically lasting 60 s or less. Redshifts are observed in Si iv, C ii, and Mg ii during the impulsive phase. Mg ii shows redshifts during the bursts and stationary emission at other times. The Si iv and C ii profiles, in contrast, are observed to be redshifted at all times during the impulsive phase. These persistent redshifts are a challenge for one-dimensional hydrodynamic models, which predict only short-duration downflows in response to impulsive heating. We conjecture that energy is being released on many small-scale filaments with a power-law distribution of heating rates.
TEMPERATURE ANISOTROPIES OF THERMAL NON-EQUILIBRIUM IONS BY A NONRESONANT ALFVÉN WAVE
Authors: Liu Hai-Feng , Tang Chang-Jian , Wang Xian-Qu , Zhang Xin , Zhao Yong 
Published: September 2016

ABSTRACT
From a significant view, considering the thermal non-equilibrium factor, we investigate Kappa (κ) ion temperature anisotropies induced by a low-frequency parallel propagating Alfvén wave by combining quasi-linear theory and test particle simulation. Analytic expressions for the ion temperature ratios

and

are derived for the solar wind, where

and

denote the perpendicular temperature of species i, parallel temperature of species i, and perpendicular temperature of species j, respectively. The results of our model are broadly consistent, compared to observations of solar-wind measurements. Solar wind helium that meets the condition for strong core heating is nearly seven times hotter than hydrogen, on average. Furthermore, we note that we are able to predict the temperature anisotropies of ions based on their thermal non-equilibrium factors.
TRACING THE REIONIZATION EPOCH WITH ALMA: [C ii] EMISSION IN z ∼ 7 GALAXIES
Authors: Pentericci L. , Carniani S. , Castellano M. , Fontana A. , Maiolino R. , Guaita L. , Vanzella E. , Grazian A. , Santini P. , Yan H. , Cristiani S. , Conselice C. , Giavalisco M. , Hathi N. , Koekemoer A. 
Published: September 2016

ABSTRACT
We present new results on [C ii]158

emission from four galaxies in the reionization epoch. These galaxies were previously confirmed to be at redshifts between 6.6 and 7.15 from the presence of the Lyα emission line in their spectra. The Lyα emission line is redshifted by 100–200 km s−1 compared to the systemic redshift given by the [C ii] line. These velocity offsets are smaller than what is observed in

Lyman break galaxies (LBGs) with similar UV luminosities and emission line properties. Smaller velocity shifts reduce the visibility of Lyα and hence somewhat alleviate the need for a very neutral intergalactic medium at

to explain the drop in the fraction of Lyα emitters observed at this epoch. The galaxies show [C ii] emission with L[C ii] =

: these luminosities place them consistently below the star formation rate (SFR)–L[C ii] relation observed for low-redshift star-forming and metal-poor galaxies and also below z = 5.5 LBGs with similar SFRs. We argue that previous undetections of [C ii] in

galaxies with similar or smaller SFRs are due to selection effects: previous targets were mostly strong Lyα emitters and therefore probably metal-poor systems, while our galaxies are more representative of the general high-redshift star-forming population.
Interchangeability between First-Line Generic Antiretroviral Products Prequalified by WHO using Adjusted Indirect Comparisons
Authors: Gwaza Luther , Gordon John , Welink Jan , Potthast Henrike , Leufkens Hubert , Stahl Matthias , García-Arieta Alfredo 
Published: September 2016

Background The scaling-up of access to antiretroviral therapy, particularly in low- to middle-income countries, was facilitated by the introduction and widespread use of generic antiretroviral medicines and fixed-dose combinations. Generic medicines are approved by regulatory authorities based on the demonstration of bioequivalence with the innovator or reference product, as well as meeting quality standards. In clinical practice, however, it is not unusual for generics to be interchanged between each other. This study investigated the differences in bioavail-ability between WHO-prequalified first-line antiretroviral generics by means of adjusted indirect comparisons to ensure interchangeability between these generics. Methods Data on 34 products containing emtricitabine, tenofovir disoproxil fumarate, lamivudine and efavirenz in single formulations or fixed-dose combinations were included in the analysis. The 90% CI for the adjusted indirect comparisons was calculated using the homoscedastic method that uses the conventional t-test, and assumes homogeneity of variances between the studies and small sample sizes. The combined standard deviation of both bioequivalence studies was calculated from the variability of each individual study. Results The adjusted indirect comparisons between generics showed that the differences, expressed as 90% CIs, are less than 30%. Confidence in the interchange-ability of two generic products was reduced if the mean difference between the test and reference in the original studies is more than 10%. Conclusions From a bioequivalence perspective, the generic antiretroviral medicines prequalified by WHO are interchangeable with the reference, as well as between each other without safety or efficacy concerns.
La captation des ressources en eau douce : la notion de dette à la lumière des marchés d’eau internationaux
Authors: Sohnle Jochen 
Published: September 2016

Quelles sont les conséquences de la captation des ressources en eau douce en droit international ? Des dettes, sont-elles créées ? Les eaux captées, peuvent-elles être mises sur un marché ? Pour répondre à ces questions, le présent article analyse d’abord quelques notions de base, à savoir celles de ressource en eau douce, de créancier, de débiteur, d’offrant et de demandeur. Deux situations principales doivent être distinguées. Si la captation s’effectue à l’intérieur d’un bassin hydrographique international ou d’un aquifère transfrontière, l’idée d’un marché de l’eau est à rejeter. En revanche, lorsqu’il y a eu une captation conforme au droit et un transfert consécutif des eaux à l’extérieur de la zone hydrographique, à l’aide d’aqueducs ou de récipients artificiels ou biologiques (eaux virtuelles), l’idée de marché est déjà un fait en droit international.
Rainfall influences performance of insecticides on the codling moth (Lepidoptera: Tortricidae) in apples
Authors: Wise John C. , Hulbert Daniel , Vandervoort Christine 
Published: September 2016

AbstractSemi-field studies were used to evaluate the effects of rainfall and field ageing on the performance of insecticides from six different chemical classes used to control the codling moth (Lepidoptera: Tortricidae). All insecticides were significantly more toxic to codling moth larvae than the untreated control, although seven-day field ageing reduced control for several compounds. Simulated rainfall events of 2.54 and 12.7 mm did not have negative effects on the performance of the insecticides. Simulated rainfall events of 50.8 mm of rainfall resulted in significant reductions of efficacy for thiacloprid and spinetoram. Residue profiles of apple leaves and fruit provided evidence for wash-off ranging from 13% to 93%, with patterns varying by compound, plant substrate, and rainfall level. This study will help apple growers make informed decisions on when reapplications of insecticides are needed in the field with the aim of improving integrated pest management.
Imbalance of the Vanin-1 Pathway in Systemic Sclerosis
Authors: Kavian Niloufar , Mehlal Souad , Marut Wioleta , Servettaz Amélie , Giessner Caroline , Bourges Christophe , Nicco Carole , Chéreau Christiane , Lemaréchal Hervé , Dutilh Marie-Flore , Cerles Olivier , Guilpain Philippe , Vuiblet Vincent , Chouzenoux Sandrine , Galland Franck , Quere Isabelle , Weill Bernard , Naquet Philippe , Batteux Frédéric 
Published: September 2016

Abstract
Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of the skin and visceral organs and vascular alterations. SSc pathophysiology involves systemic inflammation and oxidative stress. Because the vanin-1 gene (vnn1) encodes an enzyme with pantetheinase activity that converts vasculoprotective pantethine into profibrotic pantothenic acid and pro-oxidant cystamine, we tested this pathway in the pathophysiology of SSc. Activation of the vanin-1/pantetheinase pathway was investigated in wild-type BALB/c mice with hypochlorous acid (HOCl)-induced SSc by ELISA and Western blotting. We then evaluated the effects of the inactivation of vnn1 on the development of fibrosis, endothelial alterations, and immunological activation in mice with HOCl- and bleomycin-induced SSc. We then explored the vanin-1/pantetheinase pathway in a cohort of patients with SSc and in controls. In wild-type mice with HOCl-induced SSc, the vanin-1/pantetheinase pathway was dysregulated, with elevation of vanin-1 activity in skin and high levels of serum pantothenic acid. Inactivation of the vnn1 gene in vnn1−/− mice with HOCl-induced SSc prevented the development of characteristic features of the disease, including fibrosis, immunologic abnormalities, and endothelial dysfunction. Remarkably, patients with diffuse SSc also had increased expression of vanin-1 in skin and blood and elevated levels of serum pantothenic acid that correlated with the severity of the disease. Our data demonstrate that vanin-1/pantetheinase controls fibrosis, vasculopathy, autoimmunity, and oxidative stress in SSc. The levels of vanin-1 expression and pantothenic acid determine SSc severity and can be used as markers of disease severity. More importantly, inhibition of vanin-1 can open new therapeutic approaches in SSc.
Scavenger Receptor SCARA5 Acts as an HMGB1 Recognition Molecule Negatively Involved in HMGB1-Mediated Inflammation in Fish Models
Authors: Guo Dong-yang , Cao Chao , Zhang Xiao-yu , Xiang Li-xin , Shao Jian-zhong 
Published: September 2016

Abstract
Scavenger receptor class A member 5 (SCARA5) and high-mobility group box 1 (HMGB1) protein have become increasingly attractive for their critical functions in innate inflammatory reactions and disorders. However, the functional relevance between these two molecules has never been described. This study discovered that SCARA5 is an HMGB1 recognition receptor that is negatively involved in HMGB1-mediated inflammation in pufferfish (Tetraodon nigroviridis) and zebrafish (Danio rerio) models. Hence, SCARA5 is added as a new member to the HMGB1 receptor family. Tetraodon HMGB1 (TnHMGB1) is a trafficking protein that can be secreted from the nucleus to the outside of cells upon CpG-oligodeoxynucleotide (ODN) stimulation. This protein exerts a strong synergistic effect on CpG-ODN–induced inflammation, as determined by the enhanced proinflammatory cytokine expression through coadministration of TnHMGB1 with CpG-ODN and impaired inflammatory responses through TnHMGB1 depletion. Tetraodon SCARA5 (TnSCARA5) is an inducible protein detected upon TnHMGB1 stimulation; this protein plays an inhibitory role in CpG-ODN–induced inflammation because TnSCARA5 overexpression suppresses cell responsiveness to CpG-ODN induction, whereas TnSCARA5 ablation intensifies the inflammatory reactions. TnSCARA5 can strongly associate with TnHMGB1 through the A and B boxes, depending on the redox state of the cysteine residues, but T box inhibits the association. TnSCARA5 mediates the endocytosis of TnHMGB1 into lysosomes. Results suggest that TnSCARA5 inhibits the CpG-ODN–mediated inflammation via the clearance of HMGB1 mediator for CpG-ODN stimulant. The above findings highlight a novel regulatory mechanism underlying innate inflammation and provide new insights into the clinical treatment of HMGB1-mediated diseases.
Prognostic markers in smear preparations for pancreatic endocrine neoplasms: A cytomorphologic study and statistical analysis of 20 potential prognostic features
Authors: Layfield Lester J. , Schmidt Robert L. , Campbell Jack , Esebua Magda 
Published: September 2016

Background:
Papanicolaou Society of Cytopathology guidelines place low- and intermediate-grade pancreatic endocrine tumors into the “neoplastic, other” category whereas high-grade pancreatic endocrine tumors are placed in the “malignant” category. No attempt was made to stratify pancreatic endocrine tumors in the “neoplastic, other” category by likelihood for metastases. Histologically, pancreatic endocrine tumors are divided into well, intermediate, and poorly differentiated examples based on mitotic count and Ki-67 proliferation index (PI). PI has been used in the evaluation of cytologic specimens utilizing cell block material. Unfortunately, cell block material may not always be available for analysis, and little data exists as to cytomorphologic features in smear preparations which might distinguish between low- and intermediate-grade endocrine neoplasms and predict metastases.

Methods:
We studied 36 cases of Diff-Quik stained smear preparations for 20 morphologic features to determine which best-classified cases into poor and not poor outcome categories. Hierarchical logistic regression analysis was used to determine associations between the morphologic features and outcomes.

Results:
Absolute agreement between raters ranged from 51% to 97% across the 20 morphologic features. About 12 of the 20 morphologic features showed statistically significant associations with poor outcome. Mitoses, irregular nuclear membranes, and 3-fold variation in nuclear size are the best discriminators between poor and not poor outcomes.

Conclusions:
A scoring system was developed utilizing mitoses, irregular nuclear membranes, and 3-fold variation in nuclear size to divide smears of pancreatic endocrine tumors into poor and not poor outcome groups. The scoring system achieved 84% accuracy in separating cases into poor and not poor outcomes.