bluuebunny/crossref_metadata_2025 huggingface dataset:
Rapid diagnosis of Mycoplasma pneumonia infection by denaturation bubble-mediated strand exchange amplification: comparison with LAMP and real-time PCR
Authors: Shi Wenqiang , Wei Manman , Wang Qing , Wang Hongwei , Ma Cuiping , Shi Chao
Published: January 2019

M. pneumoniae infection is often ignored due to its similar clinical symptom with respiratory tract infections caused by bacteria or viruses, and thus leading to misdiagnosis and delayed treatment. It is critical to develop a rapid, sensitive and specific diagnosis method. Denaturation Bubble-mediated Strand Exchange Amplification (SEA) was established, which is an isothermal method with only a primer pair and one Bst DNA polymerase. Notably, colorimetric SEA assay was developed with simple visual readout, making instrument-independent in detection step. The method could detect as low as 1.0 × 104 copies/mL genomic DNA within 60 min. Considering that more than 80% infected patients have 1.0 × 105−1.0 × 107 copies/mL M. pneumonia DNA, SEA is available for the practical diagnosis of M. pneumoniae in clinical specimens. Through comparing 224 sputum specimens, excellent performance of SEA assay with 90.48% sensitivity and 100% specificity relative to real-time PCR was observed. Compared with LAMP, a comparable sensitivity and low false positive rate was observed for SEA method. Therefore, SEA is a promising method for detecting M. pneumoniae directly from clinical specimens, which is especially suitable for point-of-care testing in primary care facilities and resource-limited settings with minimal equipment and technological expertises.
Novel benzofuran derivative DK-1014 attenuates lung inflammation via blocking of MAPK/AP-1 and AKT/mTOR signaling in vitro and in vivo
Authors: Xu Xuezhen , Kwon Ok-Kyoung , Shin In-Sik , Mali Jyotirling R. , Harmalkar Dipesh S. , Lim Yourim , Lee Gilhye , Lu Qili , Oh Sei-Ryang , Ahn Kyung-Seop , Jeong Hye-Gwang , Lee Kyeong
Published: January 2019

Benzofuran derivatives have wide range of biological activities as anti-oxidant, anti-inflammatory and anticonvulsant agent. In this study, we investigated whether the novel benzofuran derivative, DK-1014 has the anti-inflammatory effects on macrophage and lung epithelial cells and anti-asthmatic effects on ovalbumin-treated mice. A series of 2-arylbenzofuran analogues were synthesized and evaluated for NO and interleukin-6 (IL-6) inhibition in LPS-stimulated Raw264.7 cells. Of these analogues, compounds 8, 22a, 22d, and 22 f (DK-1014) exhibited notable inhibitory activity with respect to IL-6 and NO production. In particular, compound DK-1014 strongly reduced IL-6, IL-8, and MMP-9 mRNA expression and IL-6, IL-8, and MCP-1 production in phorbol myristate acetate stimulated A549 cells, reduced MAPKs phosphorylation and c-fos translocation, and attenuated AKT, p70S6K and GSK phosphorylation. In vivo experiments were also performed on ovalbumin-sensitized and challenged BALB/c mice. DK-1014 reduced the airway hyperresponsiveness, inflammatory cell counts and cytokine levels (IL-4, 5, 13) in bronchial alveolar lavage fluid (BALF) and immunoglobulin E in serum, and attenuated inflammatory cell infiltration and mucus hypersecretion in lung tissue. These findings indicate that DK-1014 can protect against allergic airway inflammation through the AP-1 and AKT/mTOR pathways and could be useful source for the development of a therapeutic agent for asthma.
Exposure to environmental radionuclides associates with tissue-specific impacts on telomerase expression and telomere length
Authors: Kesäniemi Jenni , Lavrinienko Anton , Tukalenko Eugene , Boratyński Zbyszek , Kivisaari Kati , Mappes Tapio , Milinevsky Gennadi , Møller Anders Pape , Mousseau Timothy A. , Watts Phillip C.
Published: January 2019

Telomeres, the protective structures at the ends of chromosomes, can be shortened when individuals are exposed to stress. In some species, the enzyme telomerase is expressed in adult somatic tissues, and potentially protects or lengthens telomeres. Telomeres can be damaged by ionizing radiation and oxidative stress, although the effect of chronic exposure to elevated levels of radiation on telomere maintenance is unknown for natural populations. We quantified telomerase expression and telomere length (TL) in different tissues of the bank vole Myodes glareolus, collected from the Chernobyl Exclusion Zone, an environment heterogeneously contaminated with radionuclides, and from uncontaminated control sites elsewhere in Ukraine. Inhabiting the Chernobyl Exclusion Zone was associated with reduced TL in the liver and testis, and upregulation of telomerase in brain and liver. Thus upregulation of telomerase does not appear to associate with longer telomeres but may reflect protective functions other than telomere maintenance or an attempt to maintain shorter telomeres in a stressful environment. Tissue specific differences in the rate of telomere attrition and apparent radiosensitivity weaken the intra-individual correlation in telomere length among tissues in voles exposed to radionuclides. Our data show that ionizing radiation alters telomere homeostasis in wild animal populations in tissue specific ways.
The increase of photosynthetic carbon assimilation as a mechanism of adaptation to low temperature in Lotus japonicus
Authors: Calzadilla Pablo Ignacio , Vilas Juan Manuel , Escaray Francisco José , Unrein Fernando , Carrasco Pedro , Ruiz Oscar Adolfo
Published: January 2019

Low temperature is one of the most important factors affecting plant growth, it causes an stress that directly alters the photosynthetic process and leads to photoinhibition when severe enough. In order to address the photosynthetic acclimation response of Lotus japonicus to cold stress, two ecotypes with contrasting tolerance (MG-1 and MG-20) were studied. Their chloroplast responses were addressed after 7 days under low temperature through different strategies. Proteomic analysis showed changes in photosynthetic and carbon metabolism proteins due to stress, but differentially between ecotypes. In the sensitive MG-1 ecotype acclimation seems to be related to energy dissipation in photosystems, while an increase in photosynthetic carbon assimilation as an electron sink, seems to be preponderant in the tolerant MG-20 ecotype. Chloroplast ROS generation was higher under low temperature conditions only in the MG-1 ecotype. These data are consistent with alterations in the thylakoid membranes in the sensitive ecotype. However, the accumulation of starch granules observed in the tolerant MG-20 ecotype indicates the maintenance of sugar metabolism under cold conditions. Altogether, our data suggest that different acclimation strategies and contrasting chloroplast redox imbalance could account for the differential cold stress response of both L. japonicus ecotypes.
In silico comparison of protein-bound uremic toxin removal by hemodialysis, hemodiafiltration, membrane adsorption, and binding competition
Authors: Maheshwari Vaibhav , Thijssen Stephan , Tao Xia , Fuertinger Doris H. , Kappel Franz , Kotanko Peter
Published: January 2019

Protein-bound uremic toxins (PBUTs) are poorly removed during hemodialysis (HD) due to their low free (dialyzable) plasma concentration. We compared PBUT removal between HD, hemodiafiltration (HDF), membrane adsorption, and PBUT displacement in HD. The latter involves infusing a binding competitor pre-dialyzer, which competes with PBUTs for their albumin binding sites and increases their free fraction. We used a mathematical model of PBUT/displacer kinetics in dialysis comprising a three-compartment patient model, an arterial/venous tube segment model, and a dialyzer model. Compared to HD, improvements in removal of prototypical PBUTs indoxyl sulfate (initial concentration 100 µM, 7% free) and p-cresyl sulfate (150 µM, 5% free) were: 5.5% and 6.4%, respectively, for pre-dilution HDF with 20 L replacement fluid; 8.1% and 9.1% for post-dilution HDF 20 L; 15.6% and 18.3% for pre-dilution HDF 60 L; 19.4% and 22.2% for complete membrane adsorption; 35.0% and 41.9% for displacement with tryptophan (2000 mg in 500 mL saline); 26.7% and 32.4% for displacement with ibuprofen (800 mg in 200 mL saline). Prolonged (one-month) use of tryptophan reduces the IS and pCS time-averaged concentration by 28.1% and 29.9%, respectively, compared to conventional HD. We conclude that competitive binding can be a pragmatic approach for improving PBUT removal.
Microbiome Alteration in Type 2 Diabetes Mellitus Model of Zebrafish
Authors: Okazaki Fumiyoshi , Zang Liqing , Nakayama Hiroko , Chen Zhen , Gao Zi-Jun , Chiba Hitoshi , Hui Shu-Ping , Aoki Takahiko , Nishimura Norihiro , Shimada Yasuhito
Published: January 2019

Understanding the gut microbiota in metabolic disorders, including type 2 diabetes mellitus (T2DM), is now gaining importance due to its potential role in disease risk and progression. We previously established a zebrafish model of T2DM, which shows glucose intolerance with insulin resistance and responds to anti-diabetic drugs. In this study, we analysed the gut microbiota of T2DM zebrafish by deep sequencing the 16S rRNA V3-V4 hypervariable regions, and imputed a functional profile using predictive metagenomic tools. While control and T2DM zebrafish were fed with the same kind of feed, the gut microbiota in T2DM group was less diverse than that of the control. Predictive metagenomics profiling using PICRUSt revealed functional alternation of the KEGG pathways in T2DM zebrafish. Several amino acid metabolism pathways (arginine, proline, and phenylalanine) were downregulated in the T2DM group, similar to what has been previously reported in humans. In summary, we profiled the gut microbiome in T2DM zebrafish, which revealed functional similarities in gut bacterial environments between these zebrafish and T2DM affected humans. T2DM zebrafish can become an alternative model organism to study host-bacterial interactions in human obesity and related diseases.
Multi-omics dataset to decipher the complexity of drug resistance in diffuse large B-cell lymphoma
Authors: Fornecker Luc-Matthieu , Muller Leslie , Bertrand Frédéric , Paul Nicodème , Pichot Angélique , Herbrecht Raoul , Chenard Marie-Pierre , Mauvieux Laurent , Vallat Laurent , Bahram Seiamak , Cianférani Sarah , Carapito Raphaël , Carapito Christine
Published: January 2019

The prognosis of patients with relapsed/refractory (R/R) diffuse large B-cell lymphoma (DLBCL) remains unsatisfactory and, despite major advances in genomic studies, the biological mechanisms underlying chemoresistance are still poorly understood. We conducted for the first time a large-scale differential multi-omics investigation on DLBCL patient’s samples in order to identify new biomarkers that could early identify patients at risk of R/R disease and to identify new targets that could determine chemorefractoriness. We compared a well-characterized cohort of R/R versus chemosensitive DLBCL patients by combining label-free quantitative proteomics and targeted RNA sequencing performed on the same tissues samples. The cross-section of both data levels allowed extracting a sub-list of 22 transcripts/proteins pairs whose expression levels significantly differed between the two groups of patients. In particular, we identified significant targets related to tumor metabolism (Hexokinase 3), microenvironment (IDO1, CXCL13), cancer cells proliferation, migration and invasion (S100 proteins) or BCR signaling pathway (CD79B). Overall, this study revealed several extremely promising biomarker candidates related to DLBCL chemorefractoriness and highlighted some new potential therapeutic drug targets. The complete datasets have been made publically available and should constitute a valuable resource for the future research.
Comparative analysis of cognitive impairment prevalence and its etiological subtypes in a rural area of northern China between 2010 and 2015
Authors: Lu Hui , Wang Xiao-Dan , Shi Zhihong , Yue Wei , Zhang Ying , Liu Shuai , Liu Shuling , Zhao Lei , Xiang Lei , Zhang Yajing , Guan Yalin , Su Wenhua , Li Zhiyong , Wang Jinhuan , Wisniewski Thomas , Ji Yong
Published: January 2019

The purpose of this study was to evaluate changes in the prevalence and risk factors of cognitive impairment (CI) by analyzing and comparing two cross-sectional epidemiological surveys of CI and its subtypes were performed in a rural area of northern China between 2010 and 2015. Residents aged ≥60 years were drawn in northern China. The Mini Mental State Examination (MMSE) is recommended to test for CI. Dementia was further categorised into Alzheimer’s disease (AD), vascular dementia (VaD), and dementia caused by other diseases (ODs). Mild cognitive impairment (MCI) was classified into MCI caused by AD (MCI-A), MCI caused by VaD (MCI-VD), and MCI caused by ODs (MCI-O). The prevalence of CI increased in China. The prevalence of all-cause CI was 30·5% (22.9% MCI and 7.6% dementia) in 2010. The prevalence of all-cause CI was 38.3% (27.8% MCI and 10.5% dementia) in 2015. Similar increases were observed for the prevalence of subtypes of dementia and MCI. These findings suggest an increasing prevalence of CI and its subtypes in China, which may be related to alterations in sociodemographic factors, vascular risk factors and lifestyle changes over time in these cohorts.
Imaging study of midface growth with bone-borne trans-sutural distraction osteogenesis therapy in growing cleft lip and palate patients
Authors: Tong Haizhou , Song Tao , Sun Xiaomei , Yin Ningbei , Liu Lei , Wang Xingang , Zhao Zhenmin
Published: January 2019

Trans-sutural distraction osteogenesis (TSDO) promotes midface growth in growing cleft lip and palate (CLP) patients with midfacial hypoplasia. The superficial skeletal changes after therapy revealed rotation advancement of the midfacial skeleton associated with differential displacement in each segment, but reports rarely focus on the changes of internal structures, including circummaxillary sutures, the maxillary tuberosity and the maxillary sinus, which may play a crucial role during this process. This study evaluated the computed tomographic (CT) images of 26 growing CLP patients who received bone-borne TSDO therapy. The results revealed that the most prominent new bone formation occurred in the pterygomaxillary suture and pushed the P-point forward. The maxillary first molar exhibited significantly greater advancement compared with the P-point due to the growth of the maxillary tuberosity. The contribution ratio values of the advancement of the maxillary tuberosity and P-point to the maxillary first molar were 26% and 74%, respectively, in UCLP and 25% and 75%, respectively, in BCLP. Furthermore, the maxillary sinus volume was also significantly increased. In conclusion, midface growth with bone-borne TSDO therapy depends on both secondary displacement promoted by sutural bone formation mainly in the pterygomaxillary suture and primary displacement by growth of the maxillary tuberosity and maxillary sinus volume.
Improved Cd, Zn and Mn tolerance and reduced Cd accumulation in grains with wheat-based cell number regulator TaCNR2
Authors: Qiao Kun , Wang Fanhong , Liang Shuang , Wang Hong , Hu Zhangli , Chai Tuanyao
Published: January 2019

Soil microelement deficiency and heavy metal contamination affects plant growth and development, but improving trace element uptake and reducing heavy metal accumulation by genetic breeding can help alleviate this. Cell number regulator 2 (TaCNR2) from common wheat (Triticum aestivum) are similar to plant cadmium resistance proteins, involved with regulating heavy metal translocation. Our aim was to understand the effect of TaCNR2 on heavy metal tolerance and translocation. In this study, real-time quantitative PCR indicated TaCNR2 expression in the wheat seedlings increased under Cd, Zn and Mn treatment. Overexpression of TaCNR2 in Arabidopsis and rice enhanced its stress tolerance to Cd, Zn and Mn, and overexpression in rice improved Cd, Zn and Mn translocation from roots to shoots. The grain husks in overexpressed rice had higher Cd, Zn and Mn concentrations, but the brown rice accumulated less Cd but higher Mn than wild rice. The results showed that TaCNR2 can transport heavy metal ions. Thus, this study provides a novel gene resource for increasing nutrition uptake and reducing toxic metal accumulation in crops.
Towards reconstructing intelligible speech from the human auditory cortex
Authors: Akbari Hassan , Khalighinejad Bahar , Herrero Jose L. , Mehta Ashesh D. , Mesgarani Nima
Published: January 2019

Auditory stimulus reconstruction is a technique that finds the best approximation of the acoustic stimulus from the population of evoked neural activity. Reconstructing speech from the human auditory cortex creates the possibility of a speech neuroprosthetic to establish a direct communication with the brain and has been shown to be possible in both overt and covert conditions. However, the low quality of the reconstructed speech has severely limited the utility of this method for brain-computer interface (BCI) applications. To advance the state-of-the-art in speech neuroprosthesis, we combined the recent advances in deep learning with the latest innovations in speech synthesis technologies to reconstruct closed-set intelligible speech from the human auditory cortex. We investigated the dependence of reconstruction accuracy on linear and nonlinear (deep neural network) regression methods and the acoustic representation that is used as the target of reconstruction, including auditory spectrogram and speech synthesis parameters. In addition, we compared the reconstruction accuracy from low and high neural frequency ranges. Our results show that a deep neural network model that directly estimates the parameters of a speech synthesizer from all neural frequencies achieves the highest subjective and objective scores on a digit recognition task, improving the intelligibility by 65% over the baseline method which used linear regression to reconstruct the auditory spectrogram. These results demonstrate the efficacy of deep learning and speech synthesis algorithms for designing the next generation of speech BCI systems, which not only can restore communications for paralyzed patients but also have the potential to transform human-computer interaction technologies.
Novel carboxylate-based glycolipids: TLR4 antagonism, MD-2 binding and self-assembly properties
Authors: Cochet Florent , Facchini Fabio A. , Zaffaroni Lenny , Billod Jean-Marc , Coelho Helena , Holgado Aurora , Braun Harald , Beyaert Rudi , Jerala Roman , Jimenez-Barbero Jesus , Martin-Santamaria Sonsoles , Peri Francesco
Published: January 2019

New monosaccharide-based lipid A analogues were rationally designed through MD-2 docking studies. A panel of compounds with two carboxylate groups as phosphates bioisosteres, was synthesized with the same glucosamine-bis-succinyl core linked to different unsaturated and saturated fatty acid chains. The binding of the synthetic compounds to purified, functional recombinant human MD-2 was studied by four independent methods. All compounds bound to MD-2 with similar affinities and inhibited in a concentration-dependent manner the LPS-stimulated TLR4 signaling in human and murine cells, while being inactive as TLR4 agonists when provided alone. A compound of the panel was testedin vivoand was not able to inhibit the production of proinflammatory cytokines in animals. This lack of activity is probably due to strong binding to serum albumin, as suggested by cell experiments in the presence of the serum. The interesting self-assembly property in solution of this type of compounds was investigated by computational methods and microscopy, and formation of large vesicles was observed by cryo-TEM microscopy.
Induction of human pluripotent stem cells into kidney tissues by synthetic mRNAs encoding transcription factors
Authors: Hiratsuka Ken , Monkawa Toshiaki , Akiyama Tomohiko , Nakatake Yuhki , Oda Mayumi , Goparaju Sravan Kumar , Kimura Hiromi , Chikazawa-Nohtomi Nana , Sato Saeko , Ishiguro Keiichiro , Yamaguchi Shintaro , Suzuki Sayuri , Morizane Ryuji , Ko Shigeru B. H. , Itoh Hiroshi , Ko Minoru S. H.
Published: January 2019

The derivation of kidney tissues from human pluripotent stem cells (hPSCs) and its application for replacement therapy in end-stage renal disease have been widely discussed. Here we report that consecutive transfections of two sets of synthetic mRNAs encoding transcription factors can induce rapid and efficient differentiation of hPSCs into kidney tissues, termed induced nephron-like organoids (iNephLOs). The first set - FIGLA, PITX2, ASCL1 and TFAP2C, differentiated hPSCs into SIX2+SALL1+ nephron progenitor cells with 92% efficiency within 2 days. Subsequently, the second set - HNF1A, GATA3, GATA1 and EMX2, differentiated these cells into PAX8+LHX1+ pretubular aggregates in another 2 days. Further culture in both 2-dimensional and 3-dimensional conditions produced iNephLOs containing cells characterized as podocytes, proximal tubules, and distal tubules in an additional 10 days. Global gene expression profiles showed similarities between iNephLOs and the human adult kidney, suggesting possible uses of iNephLOs as in vitro models for kidneys.
New insights into culture negative endophthalmitis by unbiased next generation sequencing
Authors: Deshmukh Dhanshree , Joseph Joveeta , Chakrabarti Moumita , Sharma Savitri , Jayasudha Rajagopalaboopathi , Sama Kalyana C. , Sontam Bhavani , Tyagi Mudit , Narayanan Raja , Shivaji S.
Published: January 2019

The proof-of-concept, study to investigate the presence of microorganisms in presumed infectious endophthalmitis using Next generation sequencing (NGS) was carried out in vitreous biopsies from 34 patients with endophthalmitis, and thirty patients undergoing surgery for non-infectious retinal disorders as controls. Following DNA extraction using the Qiagen mini kit and PCR amplification of the V3–V4 regions of the bacterial 16S rRNA and ITS 2 region of fungus, they samples were sequenced on an Illumina HiSeq 2500 Machine. Paired reads were curated, taxonomically labeled, and filtered. Culture based diagnosis was achieved in 15/34 (44%) patients while NGS diagnosed the presence of microbes in 30/34 (88%) patients (bacteria in 26/30, fungi in 2/30, mixed infections in 2/30 cases). All 30 controls were negative for bacteria or fungus by NGS. There was good agreement between culture and NGS for culture-positive cases. Among culture negative cases, DNA of common culturable bacteria were identified like Streptococcus sp., Staphylococcus sp., Pseudomonas sp., Gemella sp., Haemophilus sp., Acinetobacter sp. The specificity of NGS with culture and clinical diagnosis was found to be 20% and 100% respectively and sensitivity of NGS with culture and clinical diagnosis was found to be 87.5% and 88% respectively. NGS appears to be promising diagnostic platform for the diagnosis of infectious culture negative endophthalmitis.
Survey of the bacteriophage phoH gene in wetland sediments in northeast China
Authors: Li Xiang , Sun Yan , Liu Junjie , Yao Qin , Wang Guanghua
Published: January 2019

PhoH is a host-derived auxiliary metabolic gene that can be used as a new biomarker for surveying phage diversity in marine and paddy waters. However, the applicability of this gene in other environments has not been addressed. In this paper, we surveyed the phoH gene in four wetland sediments in northeast China. DNA was extracted directly from sediments and used for PCR amplification with the degenerate primers vPhoHf and vPhoHr. In total, 44 and 58 phoH sequences were identified as belonging to bacteria and phages, respectively, suggesting that this primer set is not highly specific to the phage phoH gene. A BLASTp search showed that the 58 phage phoH sequences had the highest identity to the known viral sequences, ranging from 48% to 100%. Phylogenetic analysis showed that all phage sequences from wetlands distributed into the previously designated Groups 2, 3, 4 and 6. In addition, two new subgroups, Groups 2c and 4c, which contained sequences exclusively from wetlands, were detected in this study. Nonmetric multidimensional scaling analysis showed that the phage phoH assemblage from a coastal wetland was similar to that in marine environments, while the phage phoH assemblage from a lake wetland was similar to that in paddy waters. These findings indicated that different types of wetlands had distinct phage phoH compositions.
Effects of defined gut microbial ecosystem components on virulence determinants of Clostridioides difficile
Authors: Carlucci Christian , Jones Carys S. , Oliphant Kaitlyn , Yen Sandi , Daigneault Michelle , Carriero Charley , Robinson Avery , Petrof Elaine O. , Weese J. Scott , Allen-Vercoe Emma
Published: January 2019

Many cases of Clostridioides difficile infection (CDI) are poorly responsive to standard antibiotic treatment strategies, and often patients suffer from recurrent infections characterized by severe diarrhea. Our group previously reported the successful cure of two patients with recurrent CDI using a standardized stool-derived microbial ecosystem therapeutic (MET-1). Using an in vitro model of the distal gut to support bacterial communities, we characterized the metabolite profiles of two defined microbial ecosystems derived from healthy donor stool (DEC58, and a subset community, MET-1), as well as an ecosystem representative of a dysbiotic state (ciprofloxacin-treated DEC58). The growth and virulence determinants of two C. difficile strains were then assessed in response to components derived from the ecosystems. CD186 (ribotype 027) and CD973 (ribotype 078) growth was decreased upon treatment with DEC58 metabolites compared to ciprofloxacin-treated DEC58 metabolites. Furthermore, CD186 TcdA and TcdB secretion was increased following treatment with ciprofloxacin-treated DEC58 spent medium compared to DEC58 spent medium alone. The net metabolic output of C. difficile was also modulated in response to spent media from defined microbial ecosystems, although several metabolite levels were divergent across the two strains examined. Further investigation of these antagonistic properties will guide the development of microbiota-based therapeutics for CDI.
Hyperautofluorescent Dots are Characteristic in Ceramide Kinase Like-associated Retinal Degeneration
Authors: Sengillo Jesse D. , Cho Galaxy Y. , Paavo Maarjaliis , Lee Winston , White Eugenia , Jauregui Ruben , Sparrow Janet R. , Allikmets Rando , Tsang Stephen H.
Published: January 2019

There is a lack of studies which seek to discern disease expression in patients with mutations that alter retinal ceramide metabolism, specifically in the ceramide kinase like (CERKL) gene. This cross-sectional case series reports a novel phenotypic manifestation of CERKL-associated retinopathy. Four unrelated patients with homozygous CERKL mutations underwent a complete ocular exam, spectral-domain optical coherence tomography, short-wavelength fundus autofluorescence (SW-AF), quantitative autofluorescence (qAF), and full-field electroretinogram (ffERG). Decreased visual acuity and early-onset maculopathy were present in all patients. All four patients had extensive hyperautofluorescent foci surrounding an area of central atrophy on SW-AF imaging, which has not been previously characterized. An abnormal spatial distribution of qAF signal was seen in one patient, and abnormally elevated qAF8 signal in another patient. FfERG recordings showed markedly attenuated rod and cone response in all patients. We conclude that these patients exhibit several features that, collectively, may warrant screening of CERKL as a first candidate: early-onset maculopathy, severe generalized retinal dysfunction, peripheral lacunae, intraretinal pigment migration, and hyperautofluorescent foci on SW-AF.
Pharmacological cholesterol depletion disturbs ciliogenesis and ciliary function in developing zebrafish
Authors: Maerz Lars D. , Burkhalter Martin D. , Schilpp Carolin , Wittekindt Oliver H. , Frick Manfred , Philipp Melanie
Published: January 2019

Patients with an inherited inability to synthesize sufficient amounts of cholesterol develop congenital malformations of the skull, toes, kidney and heart. As development of these structures depends on functional cilia we investigated whether cholesterol regulates ciliogenesis through inhibition of hydroxymethylglutaryl-Coenzyme A reductase (HMG-CoA-R), the rate-limiting enzyme in cholesterol synthesis. HMG-CoA-R is efficiently inhibited by statins, a standard medication for hyperlipidemia. When zebrafish embryos are treated with statins cilia dysfunction phenotypes including heart defects, left-right asymmetry defects and malformation of ciliated organs develop, which are ameliorated by cholesterol replenishment. HMG-CoA-R inhibition and other means of cholesterol reduction lowered ciliation frequency and cilia length in zebrafish as well as several mammalian cell types. Cholesterol depletion further triggers an inability for ciliary signalling. Because of a reduction of the transition zone component Pi(4,5)P2 we propose that cholesterol governs crucial steps of cilium extension. Taken together, we report that cholesterol abrogation provokes cilia defects.
Direct manipulation of liquid ordered lipid membrane domains using optical traps
Authors: Friddin Mark S. , Bolognesi Guido , Salehi-Reyhani Ali , Ces Oscar , Elani Yuval
Published: January 2019

Multicomponent lipid bilayers can give rise to coexisting liquid domains that are thought to influence a host of cellular activities. There currently exists no method to directly manipulate such domains, hampering our understanding of their significance. Here we report a system that allows individual liquid ordered domains that exist in a liquid disordered matrix to be directly manipulated using optical tweezers. This allows us to drag domains across the membrane surface of giant vesicles that are adhered to a glass surface, enabling domain location to be defined with spatiotemporal control. We can also use the laser to select individual vesicles in a population to undergo mixing/demixing by locally heating the membrane through the miscibility transition, demonstrating a further layer of control. This technology has potential as a tool to shed light on domain biophysics, on their role in biology, and in sculpting membrane assemblies with user-defined membrane patterning.
Improved synthesis of β-ketoenamine-linked covalent organic frameworks via monomer exchange reactions
Authors: Daugherty Michael C. , Vitaku Edon , Li Rebecca L. , Evans Austin M. , Chavez Anton D. , Dichtel William R.
Published: January 2019

β-Ketoenamine-linked COFs with improved crystallinity are achieved through monomer exchange of isostructural imine-linked COFs.
Dual stimuli-responsive bispillar[5]arene-based nanoparticles for precisely selective drug delivery in cancer cells
Authors: Cheng Qian , Teng Kun-Xu , Ding Yuan-Fu , Yue Ludan , Yang Qing-Zheng , Wang Ruibing
Published: January 2019

Bispillar[5]arene nanoparticles exhibited dual stimuli-responsiveness towards both spermine and glutathione, allowing selective drug release in lung cancer cells.
Two novel multichromic coordination polymers based on a new flexible viologen ligand exhibiting photocontrolled luminescence properties and sensitive detection for ammonia
Authors: Wang Hai Yu , Liu Shuang , Fu Chen , Zhang Hong
Published: January 2019

Two novel Cd-CPs based on a new viologen ligand exhibiting photochromism, thermochromism, photocontrolled luminescence properties, thermocontrolled luminescence properties and ammonia detection.
Adjustable optical nonlinearity in d10 cations containing chalcogenides via dp hybridization interaction
Authors: Cheng Jianian , Lei Binghua , Zhou Chen , Pan Shilie , Yang Zhihua
Published: January 2019

The role of d10-cations on band gap and nonlinearity in Cd/Hg-containing metal chalcogenides was investigated using DFT.
The Chatt reaction: conventional routes to homoleptic arenemetalates of d-block elements
Authors: Ellis John E.
Published: January 2019

Homoleptic arenemetalates, including the depicted [K(18-crown-6)]2[Hf(C14H10)3], are often useful sources of “naked” metal anions and have been obtained for several transition metalsviathe Chatt reaction, named in honor of Joseph Chatt, a giant of chemistry.
Reduced graphene oxide-induced crystallization of CuPc interfacial layer for high performance of perovskite photodetectors
Authors: Zou Taoyu , Zhang Jianqi , Huang Shuyi , Liu Chenning , Qiu Renzheng , Wang Xiaozhi , Wu Wei , Wang Hai , Wei Zhixiang , Dai Qing , Liu Chuan , Zhang Shengdong , Zhou Hang
Published: January 2019

TS-CuPc/rGO nanocomposite thin films were synthesized and applied as an interfacial layer for high-performance perovskite-based photodiodes.
Various antibacterial mechanisms of biosynthesized copper oxide nanoparticles against soilborneRalstonia solanacearum
Authors: Chen Juanni , Mao Shuyu , Xu Zhifeng , Ding Wei
Published: January 2019

Green synthesized CuONPs disturb cell metabolism, biofilm formation, physical motility and gene expression inRalstonia solanacearum, thereby effectively controlling bacterial wilt.
Compatibility and thermal decomposition mechanism of nitrocellulose/Cr2O3 nanoparticles studied using DSC and TG-FTIR
Authors: Guo Yu , Zhao Ningning , Zhang Ting , Gong Hujun , Ma Haixia , An Ting , Zhao Fengqi , Hu Rongzu
Published: January 2019

Cr2O3 have good compatibility with nitrocellulose and catalyze decomposition of NC through decrease the activation energy. The mechanism showed Cr2O3 can accelerate the O–NO2 bond cleavage and speed up the secondary reaction.
Novel sulfonated poly(ether ether ketone)/triphenylamine hybrid membrane for vanadium redox flow battery applications
Authors: Quan Yizhou , Wang Gang , Li Anfeng , Wei Xiaoyan , Li Feng , Zhang Jie , Chen Jinwei , Wang Ruilin
Published: January 2019

The novel TPAM hybrid membrane exhibited both lower vanadium permeability and higher proton conductivity than pristine SPEEK membrane.
Cycloaddition of atmospheric CO2to epoxides under solvent-free conditions: a straightforward route to carbonates by green chemistry metrics
Authors: Monfared Aazam , Mohammadi Robab , Hosseinian Akram , Sarhandi Shahriar , Kheirollahi Nezhad Parvaneh Delir
Published: January 2019

The conversion of CO2into value-added organic compounds has received more attention over recent years since this gas is one of the major greenhouse gases, as well as an abundant, inexpensive, nontoxic, nonflammable and renewable one-carbon (C1) resource.
Synthesis and characterisation of κ2-N,O-oxazoline-enolate complexes of nickel(ii): explorations in coordination chemistry and metal-mediated polymerisation
Authors: Adjei Jeanette A. , Lough Alan J. , Gossage Robert A.
Published: January 2019

Novel Ni(ii) enolate complexes derived from (Z)-1-R-2-(4′,4′-dimethyl-2′-oxazolin-2′-yl)eth-1-en-1-ols are synthesised and structurally examined. The complexes display good potency as olefin polymerisation catalysts.
Team Approach for Education and Support of Postoperative Pediatric Cerebellar Mutism Syndrome in the Preoperative and Immediately Postoperative Phase
Authors: Molinaro Laura Hinkes , Stellpflug Wendy
Published: January 2019

Purpose

Postoperative pediatric cerebellar mutism syndrome (CMS) is a known complication following surgical resection of posterior fossa tumors. However, minimal information exists describing the progression of the syndrome and how to best support these patients. At Ann & Robert H. Lure Children's Hospital of Chicago, the brain tumor team has been fortunate enough to work together for years. Throughout the time that this particular team has been collaborating, a typical protocol has been developed for children at most risk for postoperative pediatric CMS, refined with each patient experience, to the current model. Much of what has been written about postoperative pediatric CMS (and varying terminology) has focused on potential risk factors, surgical approach, and causes, as well as definitively defining the syndrome. We would like to focus on the impact the mutism has on this constellation of symptoms. We would like to focus on the patient and family and how we as caregivers can prepare, educate, and support the family throughout this difficult diagnosis and early management of mutism.

Conclusions

There is much work to be done in describing and quantifying postoperative pediatric CMS. In the care of children with pediatric brain tumors in the preoperative and immediately postoperative phase, a team approach is paramount. When discussing expectations following surgery, consistent message and communication of hope are essential.
Performance comparison of 12/12 pole with 8/10 and 12/14 pole bearingless switched reluctance machine
Authors: Zhu Zhi‐Ying , Jiang Yong‐Jiang , Zhu Jin , Guo Xuan
Published: January 2019

In this Letter, three types of bearingless switched reluctance machines (BSRMs) with different hybrid stator structures are designed and analysed. First of all, topologies of three BSRMs, the conventional 8/10 pole, 12/14 pole BSRMs and a novel 12/12 pole BSRM with axial split phase inner stator permanent magnet structure, are given. Then, the electromagnetic performances, including the distribution of magnetic fields, suspension force and electromagnetic torque are comprehensively compared by means of 2D and 3D finite‐element method (FEM). The results based on theoretical analysis and FEM reveal that the proposed 12/12 pole BSRM would exhibit complete decoupling, shorter magnetic circuit, higher torque (power) density and suspension force than the conventional ones. Further, it is found that four degree‐of‐freedom suspension of the rotor can be realised by two‐phase suspension windings in the proposed 12/12 pole BSRM. Finally, a prototype machine is designed and manufactured to verify the validity of the topology design and electromagnetic performance.
Erosion Corrosion Behavior of Nanostructure Commercial Pure Titanium in Simulated Body Fluid
Authors: Djavanroodi F. , Irfan Osama M. , Al-Mufadi Fahad A.
Published: January 2019

To date, ECAP technique have been successfully employed to produce Ultra-fine/Nanostructure grain materials, but some materials such as hexagonal closed-packed (HCP) alloys are difficult to process by ECAP at room temperature. In this work, Transmission Electron Microscopy (TEM), Vickers hardness test and Torsion test were employed to confirm the attainment of ultrafine/nanostructured grain (UFG/NSG) commercial pure titanium (CP-Ti) Titanium fabricated by ECAP as a sever plastic deformation process. The samples were pressed by ECAP (route BC) up to four passes at elevated temperature (400° C). Finally, the Erosion-Corrosion (E-C) behavior of ultrafine/nanostructured grain (UFG/NSG) Titanium in a simulated body fluid were investigated through weight loss measurement.
Modeling the human knee joint using the Proper Generalized Decomposition
Authors: Ghnatios Chady , Hage Ilige , Metni Najib
Published: January 2019

Nowadays, human joints specifically movable are active research topics. The lack of effective replacements and the inefficient natural healing of these joints hinders any athlete from pursuing his career if injured in his joints. Therefore, researchers are testing innovative soft materials and biphasic materi- als as replacements of human joints. However, the lack of effective mechanical modeling is slowing the development of new replacements. In this work, we tackle the mechanical modeling of the synovial joint in a human knee. The tibiofemoral joint is modelled during impact. This joint is basically made of a cartilage, a meniscus (both a biphasic material) and the synovial fluid. The modeling is performed using Brinkman equation. However, the rich physics in- volved in the thickness direction requires a large number of degrees of freedom in the mesh to represent the physical phenomenon taking place in a knee joint. Thus, the use of model order reduction techniques appears to be an appealing approach in this situation. In fact, the proper generalized decomposition re- duced the number of degrees of freedom by using domain decomposition. The result of this work shows the pressure and fluid flow in the synovial joint under impact. A post treatment of the solution estimates the force held by each of the fluid and solid components of the cartilage joint. This model could be used to the human knee to estimate its components’ velocities and pressure fields while performing an activity.
Optimisation fiabiliste d’une structure en alliage à mémoire de forme
Authors: Abid Fatma , El Hami Abdelkhalak , Merzouki Tarek , Trabelsi Hassen , Walha Lassaad , Haddar Mohamed
Published: January 2019

L’objectif de l’optimisation fiabiliste (RBDO) est de trouver le meilleur compromis entre la sécurité et le coût. Plusieurs méthodes, telles que la méthode hybride (HM) et la méthode des facteurs optimaux de sûreté (OSF), sont développées pour atteindre cet objectif. Ces méthodes ont été appliquées uniquement pour les matériaux usuels. Néanmoins, le progrès effectué dans le domaine de l’élaboration de matériaux a conduit à l’utilisation des matériaux “intelligents” tel qua l’alliage à mémoire de forme (SMA). Dans ce papier, nous proposons une extension de ces méthodes dans le cas des structures formées en SMA pour étudier l’efficacité des méthodes proposées.
Bayesian updating for uncertain condition state using permanent monitoring and sequential inspections
Authors: Geara Christelle , Faddoul Rafic , Chateauneuf Alaa , Raphaël Wassim
Published: January 2019

Structural Health Monitoring using permanent sensors has been a fast growing management tool during the last decade. However, till this date, the installation of sensors on every measurable feature of the structure stills prohibitory costly. In this paper, an approach is proposed for a Bayesian updating of the condition states, based on the output of the monitoring sensors. The posterior probability distribution describing the magnitude of the defect for each element is further updated by inspecting some elements in an optimal sequence. A numerical application of the proposed methodology is presented for a concrete frame structure.
Bedform dynamics from coupled bed-flow direct numerical simulations
Authors: Zgheib N. , Fedele J.J. , Hoyal D.C.J.D. , Perillo M.M. , Balachandar S.
Published: January 2019

We present results of time-evolving coupled direct numerical simulations between an erodible bed and an overlying pressure-driven, turbulent flow field. A total of 6 simulations are considered, the details of which are shown in Table 1. The numerical setup consists of a horizontally periodic open channel, and the simulations are run at a shear Reynolds number of Reτ = 180. The coupling between the spatially and temporally evolving sediment bed and the flow field is enforced through the explicit immersed boundary method (IBM) of Uhlmann [1]. The flow field is fully resolved and is obtained by integrating the conservation of mass and momentum equations using a pseudo spectral code [2]. On the other hand, the sediment bed is modelled via the Exner equation [3]. Details about the numerical approach are available in [4-5].
Experimental investigation of the correlation between the dynamics of an impinging jet on a slotted plate and the acoustic field generated
Authors: Alkheir Marwan , Assoum Hassan , Abed-Meraïm Kamel , Mrach Tarek , Hamdi Jana , Elsoufi Louay , Skaf Hanan , Sakout Anas
Published: January 2019

In order to know the origin of the noise generation when an impinging jet hit a specific geometry, an experimental setup was used allowing the generation of the flow and the adjustments of its parameters (such as Reynolds number, confinement, alignment, etc…). The vortex dynamics in case of a high acoustic level for two different Reynolds numbers Re = 5684 and Re = 6214 are considered here. Indeed, many configurations allow self-sustaining sound loop to take place in confined spaces between the nozzle and the impinged surface. This feedback loop optimizes the energy transfer between the aerodynamic field and the acoustic field and creates a source of noise that can become very noisy. Thus, to control these phenomena, it is necessary to understand the aero-acoustic coupling in such configurations. As a result, we measured the 2C kinematic instantaneous fields (vx, vy) of the flow by the technique of Particle Image Velocimetry (PIV) with a sampling rate of 1 KHz and the acoustic field is obtained using a B&K Microphone. For the two considered Reynold numbers, we can distinguish two patterns for the vortices travelling from the nozzle toward the plate of impact.
Volumetric Proper Orthogonal Decomposition of an impinging jet using SPIV measurement
Authors: Hamdi Jana , Abed-Meraïm Kamel , Assoum Hassan , Sakout Anas , Mrach Tarek , Al-Kheir Marwan , Rambault Laurent , Cauet Sebastien , Etien Eric
Published: January 2019

Impinging jets which interact with sharp edges like slotted plates can be found in ventilation and air conditioning systems. In some cases, these configurations can be a source of desirable sounds like in musical instruments. However, they can be a source of noise in other applications such as air conditioning systems. Thus in order to reduce the noise produced from such configurations a more profound study of the flow dynamics should be implemented. All previous studies of impinging jets on a slotted plate were done in 2D. Accordingly, the objective of the present study was to reconstruct the studied volume by combining stereoscopic PIV measurements and the POD method. Consequently, an experimental set up has been developed and implemented in order to generate a flow that hits a slotted plate. On the other side, the PIV technique was used in order to study the dynamics of such a flow. The kinematic fields of twenty two parallel planes are measured by means of stereoscopic PIV. Afterwards, the analysis of the most energetic modes was obtained by the application of the snapshot POD on the kinematic fields obtained by POD. The results showed that the reconstruction of each of the planes needed only three first three modes. The reconstructed volume is obtained by an interpolation of the reconstructed planes which were phased by a trigger signal. The analysis of the vortex structures were done by the application of several vortex detection criteria.
Convection mixte MHD d’un nanofluide (eau-Cu) dans une cavité ouverte
Authors: Brahim Fersadou , Nessab Walid , Kahalerras Henda
Published: January 2019

Dans la présente étude, le problème de la convection mixte MHD d’un nanofluide (eau - Cu) confiné dans une cavité ouverte munie de deux sources de chaleur est étudié numériquement. Le modèle de Buongiorno est utilisé pour décrire l’écoulement du nanofluide en tenant compte du mouvement Brownien et de l’effet thermophorèse. Les équations gouvernantes avec les conditions aux limites associées sont résolues par la méthode des volumes finis. Les résultats révèlent un transfert de chaleur accru avec l’augmentation du rapport d’ouverture (R) et du nombre de Hartmann (Ha).
A Novel Combination Method for Image Restoration Based on Bootstrap Filter and DWT
Authors: Marhaba Bassel , Zribi Mourad
Published: January 2019

In our paper, we propose a novel combination of two methods based on the bootstrap filter. We will use the fusion technique to combine the restored images from the bootstrap multivariate kernel density filter and the bootstrap kernel-diffeomorphism filter. Experimental results have shown that our method has proved the image restoration results.