bluuebunny/crossref_metadata_2025_split huggingface dataset:
A New Glycogen Storage Disease Caused by a Dominant PYGM Mutation
Authors: Echaniz‐Laguna Andoni , Lornage Xavière , Laforêt Pascal , Orngreen Mette C. , Edelweiss Evelina , Brochier Guy , Bui Mai T. , Silva‐Rojas Roberto , Birck Catherine , Lannes Béatrice , Romero Norma B. , Vissing John , Laporte Jocelyn , Böhm Johann 
Published: May 2020

ObjectiveGlycogen storage diseases (GSDs) are severe human disorders resulting from abnormal glucose metabolism, and all previously described GSDs segregate as autosomal recessive or X‐linked traits. In this study, we aimed to molecularly characterize the first family with a dominant GSD.MethodsWe describe a dominant GSD family with 13 affected members presenting with adult‐onset muscle weakness, and we provide clinical, metabolic, histological, and ultrastructural data. We performed exome sequencing to uncover the causative gene, and functional experiments in the cell model and on recombinant proteins to investigate the pathogenic effect of the identified mutation.ResultsWe identified a heterozygous missense mutation in PYGM segregating with the disease in the family. PYGM codes for myophosphorylase, the enzyme catalyzing the initial step of glycogen breakdown. Enzymatic tests revealed that the PYGM mutation impairs the AMP‐independent myophosphorylase activity, whereas the AMP‐dependent activity was preserved. Further functional investigations demonstrated an altered conformation and aggregation of mutant myophosphorylase, and the concurrent accumulation of the intermediate filament desmin in the myofibers of the patients.InterpretationOverall, this study describes the first example of a dominant glycogen storage disease in humans, and elucidates the underlying pathomechanisms by deciphering the sequence of events from the PYGM mutation to the accumulation of glycogen in the muscle fibers. ANN NEUROL 2020;88:274–282.
Distal γ‐C(sp3)−H Olefination of Ketone Derivatives and Free Carboxylic Acids
Authors: Park Han Seul , Fan Zhoulong , Zhu Ru‐Yi , Yu Jin‐Quan 
Published: May 2020

AbstractReported herein is the distal γ‐C(sp3)−H olefination of ketone derivatives and free carboxylic acids. Fine tuning of a previously reported imino‐acid directing group and using the ligand combination of a mono‐N‐protected amino acid (MPAA) and an electron‐deficient 2‐pyridone were critical for the γ‐C(sp3)−H olefination of ketone substrates. In addition, MPAAs enabled the γ‐C(sp3)−H olefination of free carboxylic acids to form diverse six‐membered lactones. Besides alkyl carboxylic acids, benzylic C(sp3)−H bonds also could be functionalized to form 3,4‐dihydroisocoumarin structures in a single step from 2‐methyl benzoic acid derivatives. The utility of these protocols was demonstrated in large scale reactions and diversification of the γ‐C(sp3)−H olefinated products.
A Conjugated Polymer Containing Arylazopyrazole Units in the Side Chains for Field‐Effect Transistors Optically Tunable by Near Infra‐Red Light
Authors: Tian Jianwu , Liu Zitong , Jiang Wenlin , Shi Dandan , Chen Liangliang , Zhang Xisha , Zhang Guanxin , Di Chong‐an , Zhang Deqing 
Published: May 2020

AbstractOptically tunable field‐effect transistors (FETs) with near infra‐red (NIR) light show promising applications in various areas. Now, arylazopyrazole groups are incorporated in the side chains of a semiconducting donor–acceptor (D‐A) polymer. The cis–trans interconversion of the arylazopyrazole can be controlled by 980 nm and 808 nm NIR light irradiation, by utilizing NaYF4:Yb,Tm upconversion nanoparticles and the photothermal effect of conjugated D‐A polymers, respectively. This reversible transformation affects the interchain packing of the polymer thin film, which in turn reversibly tunes the semiconducting properties of the FETs by the successive 980 nm and 808 nm light irradiation. The resultant FETs display fast response to NIR light, good resistance to photofatigue, and stability in storage for up to 120 days. These unique features will be useful in future memory and bioelectronic wearable devices.
Stereoselective Synthesis of 1,1′‐Disaccharides by Organoboron Catalysis
Authors: Izumi Sanae , Kobayashi Yusuke , Takemoto Yoshiji 
Published: May 2020

AbstractThe highly stereoselective synthesis of 1,1′‐disaccharides was achieved by using 1,2‐dihydroxyglycosyl acceptors and glycosyl donors in the presence of a tricyclic borinic acid catalyst. In this reaction, the complexation of the diols and the catalyst is crucial for the activation of glycosyl donors, as well as for the 1,2‐cis‐configuration of the products. The anomeric stereochemistry of the glycosyl donor depends on the employed glycosyl donor. Applications of the produced 1,1′‐disaccharides are also described.
ACQ‐to‐AIE Transformation: Tuning Molecular Packing by Regioisomerization for Two‐Photon NIR Bioimaging
Authors: Li Yuanyuan , Liu Shunjie , Ni Huwei , Zhang Haoke , Zhang Hequn , Chuah Clarence , Ma Chao , Wong Kam Sing , Lam Jacky W. Y. , Kwok Ryan T. K. , Qian Jun , Lu Xuefeng , Tang Ben Zhong 
Published: May 2020

AbstractThe traditional design strategies for highly bright solid‐state luminescent materials rely on weakening the intermolecular π–π interactions, which may limit diversity when developing new materials. Herein, we propose a strategy of tuning the molecular packing mode by regioisomerization to regulate the solid‐state fluorescence. TBP‐e‐TPA with a molecular rotor in the end position of a planar core adopts a long‐range cofacial packing mode, which in the solid state is almost non‐emissive. By shifting molecular rotors to the bay position, the resultant TBP‐b‐TPA possesses a discrete cross packing mode, giving a quantum yield of 15.6±0.2 %. These results demonstrate the relationship between the solid‐state fluorescence efficiency and the molecule's packing mode. Thanks to the good photophysical properties, TBP‐b‐TPA nanoparticles were used for two‐photon deep brain imaging. This molecular design philosophy provides a new way of designing highly bright solid‐state fluorophores.
Triplet Acceptors with a D‐A Structure and Twisted Conformation for Efficient Organic Solar Cells
Authors: Qin Linqing , Liu Xingzheng , Zhang Xin , Yu Jianwei , Yang Lei , Zhao Fenggui , Huang Miaofei , Wang Kangwei , Wu Xiaoxi , Li Yuhao , Chen Hao , Wang Kai , Xia Jianlong , Lu Xinhui , Gao Feng , Yi Yuanping , Huang Hui 
Published: May 2020

AbstractTriplet acceptors have been developed to construct high‐performance organic solar cells (OSCs) as the long lifetime and diffusion range of triplet excitons may dissociate into free charges instead of net recombination when the energy levels of the lowest triplet state (T1) are close to those of charge‐transfer states (3CT). The current triplet acceptors were designed by introducing heavy atoms to enhance the intersystem crossing, limiting their applications. Herein, two twisted acceptors without heavy atoms, analogues of Y6, constructed with large π‐conjugated core and D‐A structure, were confirmed to be triplet materials, leading to high‐performance OSCs. The mechanism of triplet excitons were investigated to show that the twisted and D‐A structures result in large spin–orbit coupling (SOC) and small energy gap between the singlet and triplet states, and thus efficient intersystem crossing. Moreover, the energy level of T1 is close to 3CT, facilitating the split of triplet exciton to free charges.
Spatiotemporal Delivery of CRISPR/Cas9 Genome Editing Machinery Using Stimuli‐Responsive Vehicles
Authors: Cai Weiqi , Luo Tianli , Mao Lanqun , Wang Ming 
Published: May 2020

AbstractRecent innovations in genome editing have enabled the precise manipulation of the genetic information of mammalians, and benefitted the development of next‐generation gene therapy. Despite these advances, several barriers to the clinical translation of genome editing remain, including the intracellular delivery of genome editing machinery, and the risk of off‐target editing effect. Here, we review the recent advance of spatiotemporal delivery of CRISPR/Cas9 genome editing machinery, which is composed of programmable Cas9 nuclease and a single‐guide RNA (sgRNA) using stimuli‐responsive nanoparticles. We discuss the specific chemistries that have been used for controlled Cas9/sgRNA delivery and intracellular release in the presence of endogenous or external signals. These methodologies can leverage biological signals found locally within disease cells, or exogenous signals administrated with spatiotemporal control, through which an improved genome editing could be achieved. We also discuss the future in exploiting these approaches for fundamental biomedical applications and therapeutic genome editing.
Designing 3D‐MoS2Sponge as Excellent Cocatalysts in Advanced Oxidation Processes for Pollutant Control
Authors: Zhu Lingli , Ji Jiahui , Liu Jun , Mine Shinya , Matsuoka Masaya , Zhang Jinlong , Xing Mingyang 
Published: May 2020

Abstract3D‐MoS2can adsorb organic molecules and provide multidimensional electron transport pathways, implying a potential application for environment remediation. Here, we study the degradation of aromatic organics in advanced oxidation processes (AOPs) by a 3D‐MoS2sponge loaded with MoS2nanospheres and graphene oxide (GO). Exposed Mo4+active sites on 3D‐MoS2can significantly improve the concentration and stability of Fe2+in AOPs and keep the Fe3+/Fe2+in a stable dynamic cycle, thus effectively promoting the activation of H2O2/peroxymonosulfate (PMS). The degradation rate of organic pollutants in the 3D‐MoS2system is about 50 times higher than without cocatalyst. After a 140 L pilot‐scale experiment, it still maintains high efficiency and stable AOPs activity. After 16 days of continuous reaction, the 3D‐MoS2achieves a degradation rate of 120 mg L−1antibiotic wastewater up to 97.87 %. The operating cost of treating a ton of wastewater is only US$ 0.33, suggesting huge industrial applications.
Visible Light‐Induced Decarboxylative Alkylation of Heterocyclic Aromatics with Carboxylic Acids via Anthocyanin as a Photocatalyst
Authors: Guo Rui , Zuo Minghui , Tian Qinye , Hou Chuanfu , Sun Shouneng , Guo Weihao , Wu Hongfeng , Chu Wenyi , Sun Zhizhong 
Published: May 2020

AbstractA visible light‐induced decarboxylative alkylation of heterocyclic aromatics with aliphatic carboxylic acids was developed by using anthocyanins as a photocatalyst under mild conditions. A series of alkylated heterocyclic compounds were obtained in moderate to good yields by using the metal‐free decarboxylative coupling reaction under blue light. This strategy uses cheap and readily available carboxylic acids as alkylation reagents with good functional group tolerance and environmental friendliness. It is worth noting that this is the first time that anthocyanin has been used to catalyze the Minisci‐type C−H alkylation. The mechanism of decarboxylation alkylation was studied by capturing the adduct of alkyl radical and hydroquinone, thus confirming a radical mechanism. This protocol provides an alternative visible light‐induced decarboxylative alkylation for the functionalization of heterocyclic aromatics.
Techno‐economic analysis of inulooligosaccharides, protein, and biofuel co‐production from Jerusalem artichoke tubers: A biorefinery approach
Authors: Bedzo Oscar K. K. , Mandegari Mohsen , Görgens Johann F. 
Published: May 2020

AbstractJerusalem artichoke (JA) is a crop with excellent potential for application in biorefineries. It can resist drought, pests, and diseases and can thrive well in marginal lands with little fertilizer application. The JA tubers contain considerable quantities of inulin, which is suitable for the production of inulooligosaccharides (IOS), as a high‐value prebiotic, dietary fiber. In this study, five JA tuber biorefinery scenarios were simulated in Aspen Plus® and further evaluated by techno‐economic and sensitivity analyses. Production of IOS, proteins and animal feed was studied in scenarios A and C, applying various biorefinery configurations. Scenario B explored the option of producing only IOS and the sale of residues as animal feed. Scenarios D and E investigated the economic potential of biofuel generation from residues after IOS and protein production by generation of biogas and ethanol respectively, from residues. Based on the chosen economic indicators, scenario B resulted in the lowest minimum selling price (MSP) of 3.91 US$ kg−1 (market price 5.0 US$ kg−1) with correspondingly reduced total capital investment (TCI) and total operating cost (TOC) per mass unit produced of IOS of 18.91 and 2.59 US$ kg−1 respectively, compared with other studied scenarios. Considering the set production scale, it is more profitable when the residues are sold as animal feed instead of being converted into biofuel, due to the capital‐intensive nature of the biofuel production processes. The coproduction of protein had a negative impact on the economics of the process as the associated capital and operating expenditure outweighed the associated revenue. © 2020 Society of Chemical Industry and John Wiley & Sons, Ltd
Virtual reality tumor navigated robotic radical prostatectomy by using three‐dimensional reconstructed multiparametric prostate MRI and 68Ga‐PSMA PET/CT images: A useful tool to guide the robotic surgery?
Authors: Canda Abdullah Erdem , Aksoy Sertac Fatih , Altinmakas Emre , Koseoglu Ersin , Falay Okan , Kordan Yakup , Çil Barbaros , Balbay Mevlana Derya , Esen Tarik 
Published: May 2020

AbstractObjectivesTo evaluate the use and benefits of tumor navigation during performing robotic assisted radical prostatectomy (RARP).Patients and MethodsBorders of the visible tumor(s) was/were and surrounding structures marked on multiparametric prostate magnetic resonance imaging (mpMRI) and 68Ga‐labeled prostate‐specific membrane antigen ligand using positron emission computed tomography (Ga68 PSMA‐PET/CT). Three dimensional (3D) reconstruction of the images were done that were transferred to virtual reality (VR) headsets and Da Vinci surgical robot via TilePro. Images were used as a guide during RARP procedures in five cases. Indocyanine green (ICG) guided pelvic lymph node dissection (n = 2) and Martini Klinik Neurosafe technique (n = 2) were also applied.ResultsMean patient age was 60.6 ± 3.7 years (range, 56‐66). All VR models were finalized with the agreement of radiologist, urologist, nuclear physician, and engineer. Surgeon examined images before the surgery. All VR models were found very useful particularly in pT3 diseases. Pathological stages included pT2N0 (n = 1), pT3aN0 (n = 1), pT3aN1 (n = 2), and pT3bN1 (n = 1). Positive surgical margins (SMs) occurred in two patients with extensive disease (pT3aN1 and pT3bN1) and tumor occupied 30% and 50% of the prostate volumes. Mean estimated blood loss was 150 ± 86.6 cc (range, 100‐300). Mean follow‐up was 3.4 ± 1.7 months (range, 2‐6). No complication occurred during perioperative (0‐30 days) and postoperative (30‐90 days) periods in any patient.Conclusions3D reconstructed VR models by using mpMRI and Ga68 PSMA‐PET/CT images can be accurately prepared and effectively applied during RARP that might be a useful tool for tumor navigation. Images show prostate tumors and anatomy and might be a guide for the console surgeon. This is promising new technology that needs further study and validation.
Dimethyldioctadecylanimonium bentonite immobilized magnetic chitosan nanoparticles as an efficient adsorbent for vortex‐assisted magnetic dispersive micro‐solid‐phase extraction of celecoxib from human breast milk, plasma and urine samples
Authors: Majidi Seyedeh Maedeh , Hadjmohammadi Mohammad Reza 
Published: May 2020

AbstractA polymer/layered silicate composite based on dimethyldioctadecylanimonium bentonite/chitosan magnetic nanoparticles was synthesized and characterized by field emission transmission electron microscopy, X‐ray diffraction and Fourier transform infrared spectrometry. The prepared nanocomposite was used to isolate and preconcentrate celecoxib from human breast milk, urine and plasma samples. In this method, dimethyldioctadecylanimonium bentonite increases the accessibility of binding sites and adsorption capacity by high microporosity and large surface area, that has been realized for the first time in a magnetic chitosan nanoparticle support. A fractional factorial design was utilized for screening the experimental parameters. The effective parameters were then optimized by Box–Behnken design. Under the optimized conditions, the developed method exhibited wide linear ranges of 5–500 μg L−1 for plasma and urine and 10–500 μg L−1 for breast milk samples with satisfactory recoveries in the range of 96.7–99.0%. Limit of detection and quantification of celecoxib were in the ranges 0.3–3.2 and 0.99–10.56, respectively. The enrichment factors were obtained in the ranges 64.5–66.0, while precisions were <3.7%.
Uncovering the anticancer mechanism of petroleum extracts of Farfarae Flos against Lewis lung cancer by metabolomics and network pharmacology analysis
Authors: Yu Shuting , Li Jing , Gao Wei , Wu Yongyan , Qin Xuemei , Li Zhenyu 
Published: May 2020

AbstractLung cancer shows the highest incidence rate in the world. Thus, it has become increasingly important to find therapeutic drugs to treat lung cancer. Farfarae Flos (FF) has been used in traditional Chinese medicine to treat pulmonary diseases such as cough, bronchitis and asthmatic disorders. In this study, the anti‐proliferation effects of petroleum extracts of FF (PEFF) on Lewis lung cancer cells and the corresponding mechanisms were studied using cell metabolomics. Fifteen differential metabolites in the cell extracts and the corresponding medium related to the anti‐proliferation effect of PEFF were identified, which were probably involved in pyruvate metabolism and glycine, serine and threonine metabolism. For the cellular uptake compounds in PEFF, six metabolites derived from two prototype compounds were also tentatively identified by UHPLC‐Q‐Orbitrap high‐resolution MS. Network pharmacology analysis demonstrated that the anti‐proliferation mechanism of PEFF was also probably related to the target genes, including, Aurora‐A, glutathione S‐transferase Mu 1 (GSTM1), glutathione S‐transferase P 1 (GSTP1), progesterone receptor and heme oxygenase‐1 (HO‐1), and further associated with the proteoglycans and PI3K/Akt signaling pathway. Cell metabolomics and network pharmacology analysis provided a holistic method to investigate the anti‐proliferation mechanisms of PEFF. However, further studies were still needed to validate the potential target genes, pathways and active compounds in PEFF.
Enantioselective LC–ESI–MS/MS method for quantitation of (−)‐lumefantrine and (+)‐lumefantrine in mice plasma and application to a pharmacokinetic study
Authors: Kiran Vinay , Balaji Narayanan , Gabani Bhavesh Babulal , Bajantri Manjuanth , Chandran Rajesh , Dixit Abhishek , Srinivas Nuggehally R. , Mullangi Ramesh 
Published: May 2020

AbstractWe developed and validated a simple, sensitive, selective, and reliable LC–MS/MS–ESI method for the direct quantitation of lumefantrine (LFN) enantiomers [(−)‐LFN and (+)‐LFN] in mice plasma as per regulatory guideline. LFN enantiomers and carbamazepine (internal standard) were extracted from mice plasma using Strata X SPE (solid‐phase extraction) cartridges. Good resolution between enantiomers was achieved on a Chiralpak IA‐3 column using an isocratic mobile phase (0.1% of diethyl amine in methanol), which was delivered at a flow rate of 0.8 mL/min. Detection and quantitation were performed using multiple reaction monitoring mode following the transitions m/z 530.27 → 512.30 and 237.00 → 194.00 for LFN enantiomers and the internal standard, respectively, in the positive‐ionization mode. The proposed method provided accurate and reproducible results over the linearity range of 2.39–895 ng/mL for each enantiomer. The intra‐ and inter‐day precisions were in the range of 1.03–6.14 and 6.36–8.70 and 2.03–4.88 and 5.82–11.5 for (−)‐LFN and (+)‐LFN, respectively. Both (−)‐LFN and (+)‐LFN were found to be stable under different stability conditions. The method was successfully used to delineate stereoselective pharmacokinetics of LFN enantiomers in mice after an oral administration of rac‐LFN (20 mg/kg). The pharmacokinetic results indicated that the disposition of LFN enantiomers was stereoselective in mice.
Adherence with blood pressure monitoring wearable device among the elderly with hypertension: The case of rural China
Authors: Zhang Yuting , Fang Yuan , Xu Yi , Xiong Peng , Zhang Jingyi , Yang Jinru , Ran Li , Tan Xiaodong 
Published: May 2020

AbstractIntroductionWearable blood pressure (BP) monitor devices are increasingly adopted owing to the promotion of hypertension management program. However, little is known about the adherence and its associated factors in older adults (OAs) with hypertension.MethodsThe aim of this study was to determine factors associated with adherence to wearable BP monitor. In total, 212 OAs with hypertension in a remote rural area in China were asked to wear a BP monitor over 1‐month period. The following information on associated factors for adherence was collected, including demographic characteristics, cardiovascular health measurements, technology fluency, the Compliance of Hypertensive Patients' Scale, and the Health‐related Quality of Life Survey. As a result, the mean age of the 212 hypertension participants was 71.32 years (SD = 6.81).ResultsDuring the 30‐day survey, 50.94% of the participants with daily recoded BP wristband data were assigned as “BP device users.” The binary logistic regression model revealed that lower lifestyle compliance, lower medication compliance and higher total hypertension compliance were significant predictors for adherence. However, there were no differences concerning cardiovascular health factors, technology fluency, and health‐related quality of life between device users and nonusers.ConclusionIndividuals reporting a higher level of total compliance in hypertension management were more likely to wear a BP monitor device among OAs with hypertension. In addition, further research is needed to determine how wearable mHealth technology can be used to develop better hypertension self‐management education programs for this population.