bluuebunny/crossref_metadata_2025_split huggingface dataset:
Luman contributes to brefeldin A-induced prion protein gene expression by interacting with the ERSE26 element
Authors: Déry Marc-André , LeBlanc Andréa C. 
Published: February 2017

AbstractThe cellular prion protein (PrP) is essential for transmissible prion diseases, but its exact physiological function remains unclear. Better understanding the regulation of the human prion protein gene (PRNP) expression can provide insight into this elusive function. Spliced XBP1 (sXBP1) was recently shown to mediate endoplasmic reticulum (ER) stress-induced PRNP expression. In this manuscript, we identify Luman, a ubiquitous, non-canonical unfolded protein response (UPR), as a novel regulator of ER stress-induced PRNP expression. Luman activity was transcriptionally and proteolytically activated by the ER stressing drug brefeldin A (BFA) in human neurons, astrocytes, and breast cancer MCF-7 cells. Over-expression of active cleaved Luman (ΔLuman) increased PrP levels, while siRNA-mediated Luman silencing decreased BFA-induced PRNP expression. Site-directed mutagenesis and chromatin immunoprecipitation demonstrated that ΔLuman regulates PRNP expression by interacting with the ER stress response element 26 (ERSE26). Co-over-expression and siRNA-mediated silencing experiments showed that sXBP1 and ΔLuman both up-regulate ER stress-induced PRNP expression. Attempts to understand the function of PRNP up-regulation by Luman excluded a role in atorvastatin-induced neuritogenesis, ER-associated degradation, or proteasomal inhibition-induced cell death. Overall, these results refine our understanding of ER stress-induced PRNP expression and function.
CuII(atsm) improves the neurological phenotype and survival of SOD1G93A mice and selectively increases enzymatically active SOD1 in the spinal cord
Authors: Hilton James B. , Mercer Stephen W. , Lim Nastasia K. H. , Faux Noel G. , Buncic Gojko , Beckman Joseph S. , Roberts Blaine R. , Donnelly Paul S. , White Anthony R. , Crouch Peter J. 
Published: February 2017

AbstractUbiquitous expression of mutant Cu/Zn-superoxide dismutase (SOD1) selectively affects motor neurons in the central nervous system (CNS), causing the adult-onset degenerative disease amyotrophic lateral sclerosis (ALS). The CNS-specific impact of ubiquitous mutant SOD1 expression is recapitulated in transgenic mouse models of the disease. Here we present outcomes for the metallo-complex CuII(atsm) tested for therapeutic efficacy in mice expressing SOD1G93A on a mixed genetic background. Oral administration of CuII(atsm) delayed the onset of neurological symptoms, improved locomotive capacity and extended overall survival. Although the ALS-like phenotype of SOD1G93A mice is instigated by expression of the mutant SOD1, we show the improved phenotype of the CuII(atsm)-treated animals involves an increase in mature mutant SOD1 protein in the disease-affected spinal cord, where concomitant increases in copper and SOD1 activity are also evident. In contrast to these effects in the spinal cord, treating with CuII(atsm) had no effect in liver on either mutant SOD1 protein levels or its activity, indicating a CNS-selective SOD1 response to the drug. These data provide support for CuII(atsm) as a treatment option for ALS as well as insight to the CNS-selective effects of mutant SOD1.
Entorhinal Cortex dysfunction can be rescued by inhibition of microglial RAGE in an Alzheimer’s disease mouse model
Authors: Criscuolo Chiara , Fontebasso Veronica , Middei Silvia , Stazi Martina , Ammassari-Teule Martine , Yan Shirley ShiDu , Origlia Nicola 
Published: February 2017

AbstractThe Entorhinal cortex (EC) has been implicated in the early stages of Alzheimer’s disease (AD). In particular, spreading of neuronal dysfunction within the EC-Hippocampal network has been suggested. We have investigated the time course of EC dysfunction in the AD mouse model carrying human mutation of amyloid precursor protein (mhAPP) expressing human Aβ. We found that in mhAPP mice plasticity impairment is first observed in EC superficial layer and further affected with time. A selective impairment of LTP was observed in layer II horizontal connections of EC slices from 2 month old mhAPP mice, whereas at later stage of neurodegeneration (6 month) basal synaptic transmission and LTD were also affected. Accordingly, early synaptic deficit in the mhAPP mice were associated with a selective impairment in EC-dependent associative memory tasks. The introduction of the dominant-negative form of RAGE lacking RAGE signalling targeted to microglia (DNMSR) in mhAPP mice prevented synaptic and behavioural deficit, reducing the activation of stress related kinases (p38MAPK and JNK). Our results support the involvement of the EC in the development and progression of the synaptic and behavioural deficit during amyloid-dependent neurodegeneration and demonstrate that microglial RAGE activation in presence of Aβ-enriched environment contributes to the EC vulnerability.
Association evidence of CCTTT repeat polymorphism in the iNOS promoter and the risk of atrial fibrillation in Taiwanese
Authors: Hsu Lung-An , Yeh Yung-Hsin , Chen Wei-Jan , Kuo Chi-Tai , Tsai Feng-Chun , Chan Yi-Hsin , Wang Chun-Li , Chang Chi-Jen , Tsai Hsin-Yi 
Published: February 2017

AbstractInducible nitric oxide synthase (iNOS) plays an important role in the pathogenesis of atrial fibrillation (AF). The iNOS promoter has a CCTTT-repeat length polymorphism that can determine the level of gene transcription. This study enrolled 200 AF patients and 240 controls. The length of CCTTT-repeat polymorphism in the iNOS promoter region was examined by polymerase chain reactions, with the alleles with ≤11 repeats designated as S and alleles with ≥12 repeats designated as L alleles. AF patients carried significantly higher frequencies of the LL genotype than control subjects (40.0% versus 28.3%, P = 0.010). Multivariate analysis showed that the presence of LL genotype was significantly associated with AF (odds ratio: 1.87, 95% CI = 1.10–3.17, P = 0.021). In vitro, transient transfection assay in HL-1 atrial myocytes showed that the responsiveness of iNOS transcriptional activity to tachypacing was correlated with the length of the CCTTT-repeats. Right atrial tissues from patients with chronic AF were investigated with immunoconfocal microscopy. Patients with LL genotype exhibited greater oxidative stress and substrate remodeling in their atria than those with non-LL genotypes. Our results suggest that the iNOS microsatellite polymorphism may contribute to the genetic background of AF in Chinese-Taiwanese patients.
Concentration-dependent oligomerization of an alpha-helical antifreeze polypeptide makes it hyperactive
Authors: Mahatabuddin Sheikh , Hanada Yuichi , Nishimiya Yoshiyuki , Miura Ai , Kondo Hidemasa , Davies Peter L. , Tsuda Sakae 
Published: February 2017

AbstractA supersoluble 40-residue type I antifreeze protein (AFP) was discovered in a righteye flounder, the barfin plaice (bp). Unlike all other AFPs characterized to date, bpAFP transitions from moderately-active to hyperactive with increasing concentration. At sub-mM concentrations, bpAFP bound to pyramidal planes of ice to shape it into a bi-pyramidal hexagonal trapezohedron, similarly to the other moderately-active AFPs. At mM concentrations, bpAFP uniquely underwent further binding to the whole ice crystal surface including the basal planes. The latter caused a bursting ice crystal growth normal to c-axis, 3 °C of high thermal hysteresis, and alteration of an ice crystal into a smaller lemon-shaped morphology, all of which are well-known properties of hyperactive AFPs. Analytical ultracentrifugation showed this activity transition is associated with oligomerization to form tetramer, which might be the forerunner of a naturally occurring four-helix-bundle AFP in other flounders.
Spatial regularity of InAs-GaAs quantum dots: quantifying the dependence of lateral ordering on growth rate
Authors: Konishi T. , Clarke E. , Burrows C. W. , Bomphrey J. J. , Murray R. , Bell G. R. 
Published: February 2017

AbstractThe lateral ordering of arrays of self-assembled InAs-GaAs quantum dots (QDs) has been quantified as a function of growth rate, using the Hopkins-Skellam index (HSI). Coherent QD arrays have a spatial distribution which is neither random nor ordered, but intermediate. The lateral ordering improves as the growth rate is increased and can be explained by more spatially regular nucleation as the QD density increases. By contrast, large and irregular 3D islands are distributed randomly on the surface. This is consistent with a random selection of the mature QDs relaxing by dislocation nucleation at a later stage in the growth, independently of each QD’s surroundings. In addition we explore the statistical variability of the HSI as a function of the number N of spatial points analysed, and we recommend N > 103 to reliably distinguish random from ordered arrays.
VMP1-related autophagy induced by a fructose-rich diet in β-cells: its prevention by incretins
Authors: Maiztegui Bárbara , Boggio Verónica , Román Carolina L. , Flores Luis E. , Zotto Héctor Del , Ropolo Alejandro , Grasso Daniel , Vaccaro María I. , Gagliardino Juan J. 
Published: February 2017

The aim of the present study was to demonstrate the role of autophagy and incretins in the fructose-induced alteration of β-cell mass and function. Normal Wistar rats were fed (3 weeks) with a commercial diet without (C) or with 10% fructose in drinking water (F) alone or plus sitagliptin (CS and FS) or exendin-4 (CE and FE). Serum levels of metabolic/endocrine parameters, β-cell mass, morphology/ultrastructure and apoptosis, vacuole membrane protein 1 (VMP1) expression and glucose-stimulated insulin secretion (GSIS) were studied. Complementary to this, islets isolated from normal rats were cultured (3 days) without (C) or with F and F + exendin-4 or chloroquine. Expression of autophagy-related proteins [VMP1 and microtubule-associated protein light chain 3 (LC3)], apoptotic/antiapoptotic markers (caspase-3 and Bcl-2), GSIS and insulin mRNA levels were measured. F rats developed impaired glucose tolerance (IGT) and a significant increase in plasma triacylglycerols, thiobarbituric acid-reactive substances, insulin levels, homoeostasis model assessment (HOMA) for insulin resistance (HOMA-IR) and β-cell function (HOMA-β) indices. A significant reduction in β-cell mass was associated with an increased apoptotic rate and morphological/ultrastructural changes indicative of autophagic activity. All these changes were prevented by either sitagliptin or exendin-4. In cultured islets, F significantly enhanced insulin mRNA and GSIS, decreased Bcl-2 mRNA levels and increased caspase-3 expression. Chloroquine reduced these changes, suggesting the participation of autophagy in this process. Indeed, F induced the increase of both VMP1 expression and LC3-II, suggesting that VMP1-related autophagy is activated in injured β-cells. Exendin-4 prevented islet-cell damage and autophagy development. VMP1-related autophagy is a reactive process against F-induced islet dysfunction, being prevented by exendin-4 treatment. This knowledge could help in the use of autophagy as a potential target for preventing progression from IGT to type 2 diabetes mellitus.
Air and Bone Conduction Frequency-specific Auditory Brainstem Response in Children with Agenesis of the External Auditory Canal
Authors: Sleifer Pricila , Didoné Dayane , Keppeler Ísis , Bueno Claudine , Riesgo Rudimar 
Published: February 2017

Introduction The tone-evoked auditory brainstem responses (tone-ABR) enable the differential diagnosis in the evaluation of children until 12 months of age, including those with external and/or middle ear malformations. The use of auditory stimuli with frequency specificity by air and bone conduction allows characterization of hearing profile.
Objective The objective of our study was to compare the results obtained in tone-ABR by air and bone conduction in children until 12 months, with agenesis of the external auditory canal.
Method The study was cross-sectional, observational, individual, and contemporary. We conducted the research with tone-ABR by air and bone conduction in the frequencies of 500 Hz and 2000 Hz in 32 children, 23 boys, from one to 12 months old, with agenesis of the external auditory canal.
Results The tone-ABR thresholds were significantly elevated for air conduction in the frequencies of 500 Hz and 2000 Hz, while the thresholds of bone conduction had normal values in both ears. We found no statistically significant difference between genders and ears for most of the comparisons.
Conclusion The thresholds obtained by bone conduction did not alter the thresholds in children with conductive hearing loss. However, the conductive hearing loss alter all thresholds by air conduction. The tone-ABR by bone conduction is an important tool for assessing cochlear integrity in children with agenesis of the external auditory canal under 12 months.
Keratoplastiken im Kindes- und Jugendalter
Authors: Lang S. , Richter C. , Richter O. , Böhringer D. , Geerling G. , Reinhard T. 
Published: February 2017

Zusammenfassung
Hintergrund Die Voraussetzungen für das langfristige Überleben einer perforierenden Keratoplastik sind im Säuglings- und Kindesalter aufgrund immunologischer Besonderheiten, der eingeschränkten Untersuchbarkeit sowie reduzierter Compliance und Therapieadhärenz à priori ungünstig. Jedoch stellt eine Hornhautübertragung oftmals die einzige Möglichkeit zur Prävention einer Amblyopie dar und ist somit alternativlos. Wir untersuchten das Indikationsspektrum, das Transplantatüberleben und das Komplikationsspektrum in einem bizentrischen Register aller Keratoplastiken im Kindesalter.
Material und Methoden Aus den elektronischen Transplantatregistern der beiden Zentren wurden alle Patienten recherchiert, die zum Zeitpunkt der jeweils 1. Keratoplastik das 18. Lebensjahr noch nicht vollendet hatten. Aus den Krankenakten wurden die Indikation, etwaiges Transplantatversagen, die Sehschärfe, Verlust des Augapfels und weitere Komplikationen systematisch recherchiert.
Ergebnisse Es wurden insgesamt 104 Augen von 95 Patienten (54 % weiblich) identifiziert. Das Durchschnittsalter bei der Operation betrug im Median 14 Jahre (Quartile 8 und 16 Jahre). Die Nachbeobachtungszeit lag im Median bei 2,7 Jahren. Es zeigten sich folgende Indikationen: Keratokonus (39 %), perforierende Verletzung (18 %), nicht herpetische Hornhautnarben (12 %), herpetische Hornhautnarben (6 %), Sklerokornea (3 %), Verätzung (3 %) und die heterogene Gruppe der sonstigen Indikationen (19 %). Das nach Kaplan-Meier geschätzte klare Transplantatüberleben reichte zum Zeitpunkt der Durchschnittsnachbeobachtungszeit von 100 % (Keratokonus) bis 35 % (Sklerokornea). Ein Verlust des Augapfels war ausschließlich nach perforierender Verletzung (n = 2) zu verzeichnen. Nach 1 Jahr sind in der Kaplan-Meier-Schätzung alle Transplantate in der Gruppe der Säuglinge eingetrübt. In der Gruppe der Kleinkinder bis Jugendlichen sind 90 % der Transplantate zu diesem Zeitpunkt noch klar.
Schlussfolgerungen Die Prognose der Keratoplastik im Kindesalter hängt sowohl von der initialen Indikation und damit indirekt von der Grunderkrankung als auch vom Alter ab. Eine besonders schlechte Prognose zeigte die Gruppe der Säuglinge mit einem nur sehr kurzen Transplantatüberleben. Bei den Indikationen zeigte die Gruppe der Kinder mit Keratokonus die besten Ergebnisse. Als prognostisch ungünstig erwiesen sich Sklerokornea sowie perforierende Augenverletzungen.
Potenziale und Limitationen der Detektion von zirkulierenden Melanomzellen
Authors: Lüke J. , Grisanti S. , Tura A. 
Published: February 2017

ZusammenfassungDie Bestimmung und Charakterisierung zirkulierender Melanomzellen ist ein nicht invasives und komplikationsarmes Verfahren mit dem Potenzial, einen choroidalen Nävus von einem choroidalen Melanom abzugrenzen und frühzeitig einer Therapie zuführen zu können. Zukünftig bietet es die Chance, das therapeutische Ansprechen zu überwachen und bei der Therapieauswahl zu helfen. Es besteht die Hoffnung, weitere Erkenntnisse hinsichtlich des Metastasierungsprozesses zu gewinnen und damit Ansatzpunkte für die Entwicklung zielgerichteter Therapien. In diesem Artikel werden die verschiedenen Techniken der Bestimmung zirkulierender Tumorzellen vorgestellt und ihr Potenzial bzw. Limitationen diskutiert.
Nowandxianzai
Authors: Boulin Myriam 
Published: February 2017

AbstractThe present study looks at the deictic adverbsnowand 現在 (xianzai) and examines their respective roles in English and Chinese.Nowandxianzaiare commonly considered to be semantically equivalent. Their primary value is temporal: bothnowandxianzairefer to the time of utterance, or more generally to a temporal interval which includes the time of utterance. By examining a parallel corpus of Taiwanese and English novels, this paper aims to show that apart from this present-reference value,nowandxianzaihave little in common. The analysis of aligned texts reveals that: (1)nowis more frequent thanxianzai;(2) there is an asymmetry in the temporal use of both markers. FollowingWang (2001), I argue thatnowhas a wider semantic range thanxianzai: whereasnowmight be used pragmatically (Now, why on earth did she leave at such a time?),xianzaiis limited to strict temporal reference. It appears that even in its temporal use,nowis more complex thanxianzai;nowis quasi-systematically contrastive, whereasxianzaitends to be purely deictic. We conclude thatnowis closer in function to the Chinese particlelethan to the adverbxianzai.
Compounding in German and English
Authors: Berg Thomas 
Published: February 2017

Abstract
German is well-known for its propensity for nominal compounding. This claim is put on a firmer empirical footing by means of a bidirectional translation study between German and English. The difference between the two languages crystallizes in the competition between compounds and phrases. Two complementary asymmetries emerge: first, German compounds are more frequently translated by English phrases than English compounds by German phrases; second, English phrases are more frequently translated by German compounds than German phrases by English compounds. An extension to other word classes shows that the compounding bias in German is not restricted to nouns. It is tentatively argued that the token frequency of word classes plays a role in the emergence of compound propensity. The heavier use of nouns and adjectives in German than in English might be partly responsible for the higher rate of nominal and adjectival compounding in the former than the latter language.
Induced accumulation of tyramine, serotonin, and related amines in response to Bipolaris sorokiniana infection in barley
Authors: Ishihara Atsushi , Kumeda Rie , Hayashi Noriko , Yagi Yukari , Sakaguchi Nanase , Kokubo Yu , Ube Naoki , Tebayashi Shin-ichi , Ueno Kotomi 
Published: February 2017

Abstract
The inducible metabolites were analyzed in barley leaves inoculated with Bipolaris sorokiniana, the causal agent of spot blotch of barley. HPLC analysis revealed that B. sorokiniana-infected leaves accumulated 4 hydrophilic compounds. They were purified by ODS column chromatography and preparative HPLC. Spectroscopic analyses revealed that they were tyramine (1), 3-(2-aminoethyl)-3-hydroxyindolin-2-one (2), serotonin (3), and 5,5′-dihydroxy-2,4′-bitryptamine (4). Among these, 2 and 4 have not been reported as natural products. They showed antifungal activity in an assay of inhibition of B. sorokiniana conidia germination, suggesting that they play a role in the chemical defense of barley as phytoalexins. The accumulation of 1–4 was examined also in the leaves of rice and foxtail millet. Rice leaves accumulated 2, 3, and 4, whereas foxtail millet leaves accumulated 3 and 4 in response to pathogen attack, suggesting the generality of accumulation of 3 and 4 in the Poaceae species.
Postponed Feeding Does Not Substantially Reduce Production Expense during Pond Rearing of Hybrid Catfish Fry
Authors: Mischke Charles C , Griffin Matt J , Li Menghe H , Wise David J , Greenway Terrence E 
Published: February 2017

Abstract
Production variables of hybrid catfish (Channel Catfish Ictalurus punctatus × Blue Catfish I. furcatus) reared in nursery ponds and then stocked were compared between fish fed immediately after stocking (standard industry practice) and fish for which feeding was postponed after stocking. Ponds (0.04 ha) were stocked with swim-up fry at 10,000 fish/pond (250,000 fish/ha). At 2 weeks poststocking, fish in the postponed feeding treatment were significantly smaller than fish in the standard feeding protocol. Therefore, all ponds began receiving commercial diets after 2 weeks. At 2 weeks poststocking and continuing through 5 weeks poststocking, the average individual fish length and weight was greater for the standard feeding protocol than for the postponed feeding protocol. At harvest, there were no differences in production variables between the two feeding protocols. Unlike a previous study with Channel Catfish fry, where feed was withheld for 6 weeks without compromising production, noticeable deficiencies in hybrid catfish fry growth were observed after only 2 weeks of feed restriction. However, compensatory growth was observed, and postponed feeding did not result in differences in production variables observed 6 weeks poststocking. At the feeding rate and feed prices during this study, we observed feed savings of only US$38.75/ha by postponing feeding for 2 weeks.
Pedicled Sensate Composite Calcaneal Flap in Children With Congenital Tibial Pseudoarthrosis
Authors: Mongon Mauricio L.D. , Ribera Fernando C. , de Souza Antonio M.A. , Sposito Aurelio L. , Belangero William D. , Livani Bruno 
Published: February 2017

Background:
The preservation and functionality of a limb affected by a malformation (such as congenital pseudoarthrosis of the tibia) or a severely mangled lower limb in children, despite modern reconstructive techniques, remains challenging, often eventually requiring amputation to achieve a better outcome. The classical Syme and Boyd procedures are functionally better than transtibial (TT) amputation, but are not feasible for congenital tibial pseudoarthrosis. TT amputation delivers an excellent, effective, and functional stump that usually leads, after prosthetization, to a functional gait. Unfortunately, in some situations, particularly when amputation is performed conventionally, the stump is also associated with complications. Future surgical revisions are often needed, particularly in children, because of stump overgrowth.

Methods:
Between 2008 and 2010, three patients diagnosed with congenital pseudoarthrosis of the tibia associated with neurofibromatosis who were indicated for TT amputation with calcaneal flap after failure of all previous surgical reconstructive procedures were selected. The chosen method for osteosynthesis was an external fixator of Ilizarov.

Results:
At 12 weeks of follow-up, the stump had healed in all three patients, and tibiocalcaneal fusion was achieved without complications. All patients were prosthetized and had an asymptomatic gait. After a minimum follow-up of 6 years, all three cases with the pedicled sensate composite calcaneal flap still had a strong, full weight-bearing surface and have adapted easily to the conventional prosthesis, providing a painless stump with excellent functionality.

Conclusion:
With a 0 rate of needed revisions, all 3 cases with the pedicled sensate composite calcaneal flap preserving the hind foot still have a strong, full weight-bearing surface and have easily adapted to the conventional prosthesis, providing a painless and excellent functional stump that could last a lifetime.

Level of Evidence:
Level IV.