bluuebunny/crossref_metadata_2025_split huggingface dataset:
Genotype‐cardiac phenotype correlations in a large single‐center cohort of patients affected by RASopathies: Clinical implications and literature review
Authors: Leoni Chiara , Blandino Rita , Delogu Angelica Bibiana , De Rosa Gabriella , Onesimo Roberta , Verusio Valeria , Marino Maria Vittoria , Lanza Gaetano Antonio , Rigante Donato , Tartaglia Marco , Zampino Giuseppe 
Published: October 2021

AbstractCongenital heart disease (CHD) and hypertrophic cardiomyopathy (HCM) are common features in patients affected by RASopathies. The aim of this study was to assess genotype‐ phenotype correlations, focusing on the cardiac features and outcomes of interventions for cardiac conditions, in a single‐center cohort of 116 patients with molecularly confirmed diagnosis of RASopathy, and compare these findings with previously published data. All enrolled patients underwent a comprehensive echocardiographic examination. Relevant information was also retrospectively collected through the analysis of clinical records. As expected, significant associations were found between PTPN11 mutations and pulmonary stenosis (both valvular and supravalvular) and pulmonary valve dysplasia, and between SOS1 mutations and valvular defects. Similarly, HRAS mutations were significantly associated with HCM. Potential associations between less prevalent mutations and cardiac defects were also observed, including RIT1 mutations and HCM, SOS2 mutations and septal defects, and SHOC2 mutations and septal and valve abnormalities. Patients with PTPN11 mutations were the most likely to require both a primary treatment (transcatheter or surgical) and surgical reintervention. Other cardiac anomalies less reported until recently in this population, such as isolated functional and structural mitral valve diseases, as well as a sigmoid‐shaped interventricular septum in the absence of HCM, were also reported. In conclusion, our study confirms previous data but also provides new insights on cardiac involvement in RASopathies. Further research concerning genotype/phenotype associations in RASopathies could lead to a more rational approach to surgery and the consideration of drug therapy in patients at higher risk due to age, severity, anatomy, and comorbidities.
Aortic root dilatation and dilated cardiomyopathy in an adult with Tatton‐Brown‐Rahman syndrome
Authors: Cecchi Alana C. , Haidar Amier , Marin Isabella , Kwartler Callie S. , Prakash Siddharth K. , Milewicz Dianna M. 
Published: October 2021

AbstractTatton‐Brown‐Rahman syndrome is an autosomal dominant overgrowth syndrome caused by pathogenic DNMT3A variants in the germline. Clinical findings of tall stature due to postnatal overgrowth, intellectual disability, and characteristic facial features, are the most consistent findings observed in patients with Tatton‐Brown‐Rahman syndrome (TBRS). Since the syndrome was first described in 2014, an expanding spectrum of neuropsychiatric, musculoskeletal, neurological, and cardiovascular manifestations have been reported. However, most TBRS cases described in the literature are children with de novo DNMT3A variants, signaling a need to better characterize the phenotypes in adults. In this report, we describe a 34 year old referred to genetics for possible Marfan syndrome with aortic root dilatation, mitral valve prolapse, and dilated cardiomyopathy, who was diagnosed with TBRS due to a heterozygous de novo DNMT3A variant. This represents the third reported TBRS case with aortic root dilation and the second with cardiomyopathy. Collectively, these data provide evidence for an association with aortic disease and cardiomyopathy, highlight the clinical overlap with Marfan syndrome, and suggest that cardiovascular surveillance into adulthood is indicated.
On‐Surface Synthesis and Collective Spin Excitations of a Triangulene‐Based Nanostar
Authors: Hieulle Jeremy , Castro Silvia , Friedrich Niklas , Vegliante Alessio , Lara Francisco Romero , Sanz Sofía , Rey Dulce , Corso Martina , Frederiksen Thomas , Pascual Jose Ignacio , Peña Diego 
Published: October 2021

AbstractTriangulene nanographenes are open‐shell molecules with predicted high spin state due to the frustration of their conjugated network. Their long‐sought synthesis became recently possible over a metal surface. Here, we present a macrocycle formed by six [3]triangulenes, which was obtained by combining the solution synthesis of a dimethylphenyl‐anthracene cyclic hexamer and the on‐surface cyclodehydrogenation of this precursor over a gold substrate. The resulting triangulene nanostar exhibits a collective spin state generated by the interaction of its 12 unpaired π‐electrons along the conjugated lattice, corresponding to the antiferromagnetic ordering of six S=1 sites (one per triangulene unit). Inelastic electron tunneling spectroscopy resolved three spin excitations connecting the singlet ground state with triplet states. The nanostar behaves close to predictions from the Heisenberg model of an S=1 spin ring, representing a unique system to test collective spin modes in cyclic systems.
Multivariate Synergistic Flexible Metal‐Organic Frameworks with Superproton Conductivity for Direct Methanol Fuel Cells
Authors: Li Zhen‐Hua , Zeng Hui , Zeng Guang , Ru Chunyu , Li Guanghua , Yan Wenfu , Shi Zhan , Feng Shouhua 
Published: October 2021

AbstractImproving proton conductivity and fabricating viable metal‐organic frameworks (MOFs) based proton exchange membranes (PEMs) are central issues exploiting electrolyte MOFs. We aim to design multivariate flexibility synergistic strategy to achieve Flexible MOFs (FMOFs) with high conductivity at a wide range of humidity. In situ powder X‐ray diffraction (PXRD) and temperature‐dependent Fourier transform infrared spectra (FT‐IR) prove the synergistic self‐adaption between dynamic torsion of alkyl sulfonic acid and dynamic breathing of FMOF, forming a continuous hydrogen‐bonding networks to maintain high conductivity. Based on the convincing proton conductivity, we construct a series of long‐term durable MOF‐based PEMs that serve as a bridge between MOF and fuel cell. Consequently, the membrane electrode assembly (MEA) of the flexible PMNS1‐40 exhibits a maximum single‐cell power density of 34.76 mW cm−2 and hopefully opens doors to evaluate the practical application of proton‐conducting MOFs in direct methanol fuel cells.
Copper‐Catalyzed Covalent Dimerization of Near‐Infrared Fluorescent Cyanine Dyes: Synergistic Enhancement of Photoacoustic Signals for Molecular Imaging of Tumors
Authors: Maji Dolonchampa , Oh Donghyeon , Sharmah Gautam Krishna , Zhou Mingzhou , Zhang Haini , Kao Jeff , Giblin Daryl , Smith Matthew , Lim Junha , Lee Seunghyun , Kang Youngnam , Kim Won Jong , Kim Chulhong , Achilefu Samuel 
Published: October 2021

AbstractPhotoacoustic (PA) imaging relies on the absorption of light by chromophores to generate acoustic waves used to delineate tissue structures and physiology. Here, we demonstrate that Cu(II) efficiently catalyzes the dimerization of diverse near‐infrared (NIR) cyanine molecules, including a peptide conjugate. NMR spectroscopy revealed a C−C covalent bond along the heptamethine chains, creating stable molecules under conditions such as a wide range of solvents and pH mediums. Dimerization achieved >90 % fluorescence quenching, enhanced photostability, and increased PA signals by a factor of about 4 at equimolar concentrations compared to the monomers. In vivo study with a mouse cancer model revealed that dimerization enhanced tumor retention and PA signal, allowing cancer detection at doses where the monomers are less effective. While the dye dimers highlighted peritumoral blood vessels, the PA signal for dimeric tumor‐targeting dye‐peptide conjugate, LS301, was diffuse throughout the entire tumor mass. A combination of the ease of synthesis, diversity of molecules that are amenable to Cu(II)‐catalyzed dimerization, and the high acoustic wave amplification by these stable dimeric small molecules ushers a new strategy to develop clinically translatable PA molecular amplifiers for the emerging field of molecular photoacoustic imaging.
Predicting aquatic invasions in a megadiverse region: Maximum‐entropy‐based modelling of six alien fish species in Malaysia
Authors: Vythalingam Lavanya Malini , Raghavan Rajeev , Hossain Motalib , Bhassu Subha 
Published: October 2021

Abstract

There has been a recent increase in the occurrence and proliferation of alien fish species in rivers, lakes, and reservoirs of Malaysia, posing a significant risk to native biodiversity. This increase has been largely caused by the unmanaged aquaculture and aquarium pet trade.
At present, very little information is available on the diversity and distribution of alien fish species in freshwater ecosystems of Malaysia, as well as their current and future threats.
Using the MaxEnt model, the potential habitat suitability and future invasion ranges of six alien fish species (Cichla ocellaris, Clarias gariepinus, Oreochromis mossambicus, Oreochromis niloticus, Poecilia reticulata, and Pterygoplichthys pardalis) in Peninsular and East Malaysia (Borneo) were determined.
All six alien fish species investigated, have high potential to invade eight states in Peninsular Malaysia (Johor, Kelantan, Malacca, Negeri Sembilan, Pahang, Perak, Selangor, and Terengganu), and much of East Malaysia. The results also indicate that alien fish are likely to invade the majority of the 444 protected areas in the country, particularly those located in Sabah and Sarawak, which are known for their high fish diversity and endemism.
Minimum temperature of the coldest month (Bio 6) and isothermality (Bio 3) significantly influence potential invasion habitats for all six alien fish species in Malaysia. The country's hot and humid climate makes it ideal for the survival and proliferation of alien species except for P. pardalis.
These findings will help conservation planners and managers in identifying areas of high concern for alien freshwater fish species in Malaysia, and to pre‐emptively mitigate and manage their future invasion.
Tailoring the Pore Size, Basicity, and Binding Energy of Mesoporous C3N5 for CO2 Capture and Conversion
Authors: Kim Sungho , Singh Gurwinder , Sathish CI , Panigrahi Puspamitra , Daiyan Rahman , Lu Xunyu , Sugi Yoshihiro , Kim In Young , Vinu Ajayan 
Published: October 2021

AbstractWe investigated the CO2 adsorption and electrochemical conversion behavior of triazole‐based C3N5 nanorods as a single matrix for consecutive CO2 capture and conversion. The pore size, basicity, and binding energy were tailored to identify critical factors for consecutive CO2 capture and conversion over carbon nitrides. Temperature‐programmed desorption (TPD) analysis of CO2 demonstrates that triazole‐based C3N5 shows higher basicity and stronger CO2 binding energy than g‐C3N4. Triazole‐based C3N5 nanorods with 6.1 nm mesopore channels exhibit better CO2 adsorption than nanorods with 3.5 and 5.4 nm mesopore channels. C3N5 nanorods with wider mesopore channels are effective in increasing the current density as an electrocatalyst during the CO2 reduction reaction. Triazole‐based C3N5 nanorods with tailored pore sizes exhibit CO2 adsorption abilities of 5.6–9.1 mmol/g at 0 °C and 30 bar. Their Faraday efficiencies for reducing CO2 to CO are 14–38% at a potential of −0.8 V vs. RHE.
Improved operational reliability and contaminant removal in water reuse through filter upgrades
Authors: Stanford Benjamin D. , Arrington D. Albrey , Raseman William J. , Becker William C. 
Published: October 2021

AbstractA redesign, construction, and replacement of the filters used for water reuse at a 11 million gallon per day Florida municipal water reclamation facility provided a prime opportunity to evaluate the impact of the filtration technologies on water quality, trace organic contaminant (TOrC) removal, and removal of pathogens. This study was designed to capture operational data, pathogen removal (Cryptosporidium and Giardia), and TOrC removal before the replacement of the filters using the existing synthetic media filters (SMF) and traveling bridge filters (TBF) followed by an evaluation of the deep bed filters (DBF) after construction and commissioning. The new DBF units provided substantially improved control of turbidity and total suspended solids while also significantly improving the removal of Giardia cysts (increase of >2 log removal) as compared to removal across the older SMF and TBF units and minor improvements to Cryptosporidium oocyst removal. TOrC removal was not significantly changed when comparing removal across the SMF, TBF, and DBF units nor post chlorination (chloramination).Article Impact StatementThis manuscript will be of use to the community as it provides additional understanding of public health protection in water reuse.
Biochanin‐A has antidiabetic, antihyperlipidemic, antioxidant, and protective effects on diabetic nephropathy via suppression of TGF‐β1 and PAR‐2 genes expression in kidney tissues of STZ‐induced diabetic rats
Authors: Amri Jamal , Alaee Mona , Babaei Rasool , Salemi Zahra , Meshkani Reza , Ghazavi Ali , Akbari Ahmad , Salehi Mehdi 
Published: October 2021

AbstractOne of the major complications of diabetes is diabetic nephropathy, and often many patients suffer from diabetic nephropathy. That is why it is important to find the mechanisms that cause nephropathy and its treatment. This study was designed to examine the antidiabetic effects of biochanin A (BCA) and evaluate its effects on oxidative stress markers and the expression of transforming growth factor‐β1 (TGF‐β1) and protease‐activated receptors‐2 (PAR‐2) genes in the kidney of type 1 diabetic rats. After induction of diabetes using streptozotocin (STZ), 55 mg/kg bw dose, rats were randomly divided into four groups with six rats in each group as follows: normal group: normal control receiving normal saline and a single dose of citrate buffer daily; diabetic control group: diabetic control receiving 0.5% dimethyl sulfoxide daily; diabetic+BCA (10 mg/kg) group: diabetic rats receiving biochanin A at a dose of 10 mg/kg bw daily; diabetic+BCA (15 mg/kg) group: diabetic rats receiving biochanin A at a dose of 15 mg/kg bw daily. TGF‐β1 and PAR‐2 gene expression was assessed by real‐time. Spectrophotometric methods were used to measure biochemical factors: fast blood glucose (FBG), urea, creatinine, albumin, lipids profiles malondialdehyde (MDA), and superoxide dismutase (SOD). The course of treatment in this study was 42 days. The results showed that in the diabetic control group, FBG, serum urea, creatinine, expression of TGF‐β1 and PAR‐2 genes, and the levels of MDA in kidney tissue significantly increased and SOD activity in kidney tissue and serum albumin significantly decreased compared to the normal group (p < 0.001). The results showed that administration of biochanin A (10 and 15 mg/kg) after 42 days significantly reduced the expression of TGF‐β1 and PAR‐2 genes and FBG, urea, creatinine in serum compared to the diabetic control group (p < 0.001), also significantly increased serum albumin compared to the diabetic control group (p < 0.001). The level of MDA and SOD activity in the tissues of diabetic rats that used biochanin A (10 and 15 mg/kg) was significantly reduced and increased, respectively, compared to the diabetic control group (p < 0.001). Also, the result showed that in the diabetic control group lipids profiles significantly is disturbed compared to the normal group (p < 0.001), the results also showed that biochanin A (10 and 15 mg/kg) administration could significantly improved the lipids profile compared to the control diabetic group (p < 0.001). It is noteworthy that it was found that the beneficial effects of the biochanin A were dose dependent. In conclusion, administration of biochanin A for 42 days has beneficial effect and improves diabetes and nephropathy in diabetic rats. So probably biochanin A can be used as an adjunct therapy in the treatment of diabetes.
The US bioeconomy at the intersection of technology, policy, and education
Authors: Pascoli Danielle U , Aui Alvina , Frank Jenny , Therasme Obste , Dixon Kerry , Gustafson Rick , Kelly Brendan , Volk Timothy A , Wright Mark M 
Published: October 2021

AbstractThe bioeconomy is a complex, multivariate, and interdisciplinary system that requires a comprehensive assessment of its independent parts if it is to be understood fully. Hence, this article presents a holistic perspective of industry, public policy, and education aspects of the US bioeconomy. It is premised on the idea that a successful bioeconomy industry relies on the balanced development of all stages of the supply chain. For this balance to be struck, a strong interdisciplinary workforce must find novel solutions to multifaceted problems across the entirety of the supply chain. These solutions require innovative technologies that can improve the climate benefit of bioproducts, decrease their production costs, and make them more economically competitive. Increasing consumer education and awareness about the bioeconomy goes hand in hand with the development of a robust market for bioproducts. To guide these interdependent efforts, public policies must encourage demand, support competitive markets, promote the entry of renewable options, and stimulate growth by reducing financial barriers. We contend that a combination of policies is likely to be more effective than any singular policy on its own. Supporting the bioeconomy also entails attending to an existing lack of public awareness as well as workforce‐ready professionals. To address these gaps, the USA must increase the intensity and intentionality of its efforts to educate students about the bioeconomy, particularly at the K‐12 level. Furthermore, these efforts should encompass both formal and informal learning contexts in order to meet the workforce challenges facing the bioeconomy now and in the future. © 2021 The Authors. Biofuels, Bioproducts and Biorefining published by Society of Industrial Chemistry and John Wiley & Sons Ltd.
Improvement of the performance of anticancer peptides using a drug repositioning pipeline
Authors: Mohammadi Elyas , Tahmoorespur Mojtaba , Benfeitas Rui , Altay Ozlem , Javadmanesh Ali , Lam Simon , Mardinoglu Adil , Sekhavati Mohammad Hadi 
Published: October 2021

AbstractThe use of anticancer peptides (ACPs) as an alternative/complementary strategy to conventional chemotherapy treatments has been shown to decrease drug resistance and/or severe side effects. However, the efficacy of the positively‐charged ACP is inhibited by elevated levels of negatively‐charged cell‐surface components which trap the peptides and prevent their contact with the cell membrane. Consequently, this decreases ACP‐mediated membrane pore formation and cell lysis. Negatively‐charged heparan sulphate (HS) and chondroitin sulphate (CS) have been shown to inhibit the cytotoxic effect of ACPs.In this study, we propose a strategy to promote the broad utilization of ACPs. In this context, we developed a drug repositioning pipeline to analyse transcriptomics data generated for four different cancer cell lines (A549, HEPG2, HT29, and MCF7) treated with hundreds of drugs in the LINCS L1000 project. Based on previous studies identifying genes modulating levels of the glycosaminoglycans (GAGs) HS and CS at the cell surface, our analysis aimed at identifying drugs inhibiting genes correlated with high HS and CS levels. As a result, we identified six chemicals as likely repositionable drugs with the potential to enhance the performance of ACPs. The codes in R and Python programming languages are publicly available inhttps://github.com/ElyasMo/ACPs_HS_HSPGs_CS.As a conclusion, these six drugs are highlighted as excellent targets for synergistic studies with ACPs aimed at lowering the costs associated with ACP‐treatment.
Development of an E. coli strain for cell‐free ADC manufacturing
Authors: Groff Dan , Carlos Nina A. , Chen Rishard , Hanson Jeffrey A. , Liang Shengwen , Armstrong Stephanie , Li Xiaofan , Zhou Sihong , Steiner Alex , Hallam Trevor J. , Yin Gang 
Published: October 2021

AbstractRecent advances in cell‐free protein synthesis have enabled the folding and assembly of full‐length antibodies at high titers with extracts from prokaryotic cells. Coupled with the facile engineering of the Escherichia coli translation machinery, E. coli based in vitro protein synthesis reactions have emerged as a leading source of IgG molecules with nonnatural amino acids incorporated at specific locations for producing homogeneous antibody–drug conjugates (ADCs). While this has been demonstrated with extract produced in batch fermentation mode, continuous extract fermentation would facilitate supplying material for large‐scale manufacturing of protein therapeutics. To accomplish this, the IgG‐folding chaperones DsbC and FkpA, and orthogonal tRNA for nonnatural amino acid production were integrated onto the chromosome with high strength constitutive promoters. This enabled co‐expression of all three factors at a consistently high level in the extract strain for the duration of a 5‐day continuous fermentation. Cell‐free protein synthesis reactions with extract produced from cells grown continuously yielded titers of IgG containing nonnatural amino acids above those from extract produced in batch fermentations. In addition, the quality of the synthesized IgGs and the potency of ADC produced with continuously fermented extract were indistinguishable from those produced with the batch extract. These experiments demonstrate that continuous fermentation of E. coli to produce extract for cell‐free protein synthesis is feasible and helps unlock the potential for cell‐free protein synthesis as a platform for biopharmaceutical production.
Development of surface‐enhanced Raman scattering‐based immunoassay platforms using hollow Au nanostars for reliable SARS‐CoV‐2 diagnosis
Authors: Yu Qian , Wu Yixuan , Kang Taejoon , Choo Jaebum 
Published: October 2021

AbstractWe developed a novel surface‐enhanced Raman scattering (SERS)‐based SARS‐CoV‐2 assay platform using hollow Au nanostars to realize high‐sensitivity diagnosis of SARS‐CoV‐2. The assay was performed using SARS‐CoV‐2 lysate as the target in a wide dynamic range with virus concentrations ranging from 0 to 104 PFU/ml and has a limit of detection (LOD) of 5.1 PFU/ml. This LOD value shows 100 times and 10 times better sensitivity compared to the LODs measured on the same sample using a commercially available rapid kit and enzyme‐linked immunosorbent assay, respectively. Therefore, we believe that this SERS‐based SARS‐CoV‐2 assay platform has high diagnostic accuracy for early or asymptomatic infected patients with low virus concentrations. Furthermore, the probability of a false‐negative diagnosis is likely to be very low.
Volatile Nitrogenous Compounds from Bacteria: Source of Novel Bioactive Compounds
Authors: Valença Camilla A. S. , Barbosa Ana A. T. , Souto Eliana B. , Caramão Elina B. , Jain Sona 
Published: October 2021

AbstractBacteria can produce nitrogenous compounds via both primary and secondary metabolic processes. Many bacterial volatile nitrogenous compounds produced during the secondary metabolism have been identified and reported for their antioxidant, antibacterial, antifungal, algicidal and antitumor activities. The production of these nitrogenous compounds depends on several factors, including the composition of culture media, growth conditions, and even the organic solvent used for their extraction, thus requiring their identification in specific conditions. In this review, we describe the volatile nitrogenous compounds produced by bacteria especially focusing on their antimicrobial activity. We concentrate on azo‐compounds mainly pyrazines and pyrrolo‐pyridines reported for their activity against several microorganisms. Whenever significant, extraction and identification methods of these compounds are also mentioned and discussed. To the best of our knowledge, this is first review describing volatile nitrogenous compounds from bacteria focusing on their biological activity.