bluuebunny/crossref_metadata_2025_split huggingface dataset:
Structural brain anomalies in patients with FOXG1 syndrome and in Foxg1+/− mice
Authors: Pringsheim Milka , Mitter Diana , Schröder Simone , Warthemann Rita , Plümacher Kim , Kluger Gerhard , Baethmann Martina , Bast Thomas , Braun Sarah , Büttel Hans‐Martin , Conover Elizabeth , Courage Carolina , Datta Alexandre N. , Eger Angelika , Grebe Theresa A. , Hasse‐Wittmer Annette , Heruth Marion , Höft Karen , Kaindl Angela M. , Karch Stephanie , Kautzky Torsten , Korenke Georg C. , Kruse Bernd , Lutz Richard E. , Omran Heymut , Patzer Steffi , Philippi Heike , Ramsey Keri , Rating Tina , Rieß Angelika , Schimmel Mareike , Westman Rachel , Zech Frank‐Martin , Zirn Birgit , Ulmke Pauline A. , Sokpor Godwin , Tuoc Tran , Leha Andreas , Staudt Martin , Brockmann Knut 
Published: March 2019

AbstractObjectiveFOXG1 syndrome is a rare neurodevelopmental disorder associated with heterozygous FOXG1 variants or chromosomal microaberrations in 14q12. The study aimed at assessing the scope of structural cerebral anomalies revealed by neuroimaging to delineate the genotype and neuroimaging phenotype associations.MethodsWe compiled 34 patients with a heterozygous (likely) pathogenic FOXG1 variant. Qualitative assessment of cerebral anomalies was performed by standardized re‐analysis of all 34 MRI data sets. Statistical analysis of genetic, clinical and neuroimaging data were performed. We quantified clinical and neuroimaging phenotypes using severity scores. Telencephalic phenotypes of adult Foxg1+/− mice were examined using immunohistological stainings followed by quantitative evaluation of structural anomalies.ResultsCharacteristic neuroimaging features included corpus callosum anomalies (82%), thickening of the fornix (74%), simplified gyral pattern (56%), enlargement of inner CSF spaces (44%), hypoplasia of basal ganglia (38%), and hypoplasia of frontal lobes (29%). We observed a marked, filiform thinning of the rostrum as recurrent highly typical pattern of corpus callosum anomaly in combination with distinct thickening of the fornix as a characteristic feature. Thickening of the fornices was not reported previously in FOXG1 syndrome. Simplified gyral pattern occurred significantly more frequently in patients with early truncating variants. Higher clinical severity scores were significantly associated with higher neuroimaging severity scores. Modeling of Foxg1 heterozygosity in mouse brain recapitulated the associated abnormal cerebral morphology phenotypes, including the striking enlargement of the fornix.InterpretationCombination of specific corpus callosum anomalies with simplified gyral pattern and hyperplasia of the fornices is highly characteristic for FOXG1 syndrome.
Investigation of white matter PiB uptake as a marker of white matter integrity
Authors: Zeydan Burcu , Schwarz Christopher G. , Lowe Val J. , Reid Robert I. , Przybelski Scott A. , Lesnick Timothy G. , Kremers Walter K. , Senjem Matthew L. , Gunter Jeffrey L. , Min Hoon‐Ki , Vemuri Prashanthi , Knopman David S. , Petersen Ronald C. , Jack Clifford R. , Kantarci Orhun H. , Kantarci Kejal 
Published: March 2019

AbstractObjectiveTo investigate the associations of Pittsburgh compound‐B (PiB) uptake in white matter hyperintensities (WMH) and normal appearing white matter (NAWM) with white matter (WM) integrity measured with DTI and cognitive function in cognitively unimpaired older adults.MethodsCognitively unimpaired older adults from the population‐based Mayo Clinic Study of Aging (n = 537, age 65–95) who underwent both PiB PET and DTI were included. The associations of WM PiB standard uptake value ratio (SUVr) with fractional anisotropy (FA) and mean diffusivity (MD) in the WMH and NAWM were tested after adjusting for age. The associations of PiB SUVr with cognitive function z‐scores were tested after adjusting for age and global cortical PiB SUVr.ResultsThe WMH PiB SUVr was lower than NAWM PiB SUVr (P < 0.001). In the WMH, lower PiB SUVr correlated with lower FA (r = 0.21, P < 0.001), and higher MD (r = −0.31, P < 0.001). In the NAWM, lower PiB SUVr only correlated with higher MD (r = −0.10, P = 0.02). Both in the WMH and NAWM, lower PiB SUVr was associated with lower memory, language, and global cognitive function z‐scores after adjusting for age and global cortical PiB SUVr.InterpretationReduced PiB uptake in the WMH is associated with a loss of WM integrity and cognitive function after accounting for the global cortical PiB uptake, suggesting that WM PiB uptake may be an early biomarker of WM integrity that precedes cognitive impairment in older adults. When using WM as a reference region in cross‐sectional analysis of PiB SUVr, individual variability in WMH volume as well as age should be considered.
Toll‐like receptor‐4 299Gly allele is associated with Guillain‐Barré syndrome in Bangladesh
Authors: Jahan Israt , Ahammad Rijwan U. , Khalid Mir M. , Rahman Mohammad I. , Hayat Shoma , Islam Badrul , Mohammad Quazi D. , Islam Zhahirul 
Published: March 2019

AbstractObjectiveTLR4 plays an important role in the pathogenesis of Guillain‐Barré syndrome (GBS). The relationships between TLR4 polymorphisms and susceptibility to GBS are poorly understood. We investigated the frequency and assessed the association of two single nucleotide polymorphisms (SNPs) in the extracellular domain of TLR4 (Asp299Gly and Thr399Ile) with disease susceptibility and the clinical features of GBS in a Bangladeshi cohort.MethodsA total of 290 subjects were included in this study: 141 patients with GBS and 149 unrelated healthy controls. The TLR4 polymorphisms Asp299Gly and Thr399Ile were genotyped using polymerase chain reaction‐restriction fragment length polymorphism (PCR‐RFLP) assay.ResultsThe minor 299Gly allele was significantly associated with GBS susceptibility (P = 0.0137, OR = 1.97, 95% CI = 1.17–3.31), and was present at a significantly higher frequency in patients with the acute motor axonal neuropathy (AMAN) subtype of GBS (P = 0.0120, OR = 2.37, 95% CI = 1.26–4.47) than acute inflammatory demyelinating polyneuropathy (AIDP) subtype (P = 0.961, OR = 1.15, 95% CI = 0.38–3.48); when compared to healthy controls. The genotype frequency of the Asp299Gly polymorphism was not significantly different between patients with GBS and healthy controls. The Asp299‐Thr399 haplotype was associated with a significantly lower risk of developing GBS (P = 0.0451, OR = 0.63, 95% CI = 0.40–0.99). No association was observed between the Thr399Ile polymorphism and GBS disease susceptibility.InterpretationThe TLR4 minor 299Gly allele was associated with increased susceptibility to GBS and the axonal GBS subtype in the Bangladeshi population. However, no associations were observed between the genotypes of the Asp299Gly and Thr399Ile SNPs and antecedent C. jejuni infection or disease severity in Bangladeshi patients with GBS.
In Situ Generated Medical Devices
Authors: Cohn Daniel , Sloutski Aaron , Elyashiv Ariel , Varma Vijaykumar B. , Ramanujan Raju 
Published: March 2019

AbstractMedical devices play a major role in all areas of modern medicine, largely contributing to the success of clinical procedures and to the health of patients worldwide. They span from simple commodity products such as gauzes and catheters, to highly advanced implants, e.g., heart valves and vascular grafts. In situ generated devices are an important family of devices that are formed at their site of clinical function that have distinct advantages. Among them, since they are formed within the body, they only require minimally invasive procedures, avoiding the pain and risks associated with open surgery. These devices also display enhanced conformability to local tissues and can reach sites that otherwise are inaccessible. This review aims at shedding light on the unique features of in situ generated devices and to underscore leading trends in the field, as they are reflected by key developments recently in the field over the last several years. Since the uniqueness of these devices stems from their in situ generation, the way they are formed is crucial. It is because of this fact that in this review, the medical devices are classified depending on whether their in situ generation entails chemical or physical phenomena.
Substrate‐Independent Coating with Persistent and Stable Antifouling and Antibacterial Activities to Reduce Bacterial Infection for Various Implants
Authors: Yuan Pingyun , Qiu Xinyu , Wang Xinran , Tian Ran , Wang Lin , Bai Yongkang , Liu Shiyu , Chen Xin 
Published: March 2019

AbstractImplantation of biomedical devices accompanying infections has caused severe problems to public health that require feasible solutions. In this study, a simple approach is reported to fabricate a antimicrobial and antifouling dual‐functional coating. This coating consists of a substrate‐independent layer‐by‐layer (LBL) film formed by poly (diallyldimethylammonium) (PDDA) and poly (styrenesulfonate) (PSS), where parts of PSS and PDDA are physically substituted by hetero‐bifunctional polyethylene glycol (PEG) ending with a carboxyl group and antimicrobial peptide (ε‐Poly‐l‐lysine, ε‐PL). This design (ε‐PL‐PEG‐(PDDA/PSS)9 coating) exhibits not only potent antimicrobial activity against Gram‐positive/negative bacteria but also superior antifouling activity on various substrates, including glass and plastic. Moreover, the antifouling and antibacterial performance can be maintained for a longer period of time under physiological environments even after physical damage of the surface due to the homogeneous interspersion and free migration of ε‐PL‐PEG‐COOH in the LBL film. This allows the supplement of these molecules to the surface against molecule loss during usage. Both in vitro and in vivo (rodent subcutaneous infection model) studies show obvious reduction of the bacteria on the coated substrate and in the surrounding tissues with up to 3.2‐log reduction, even after repeated usage. The inflammation around the implantation area is also significantly inhibited.
Building Reconfigurable Devices Using Complex Liquid–Fluid Interfaces
Authors: Forth Joe , Kim Paul Y. , Xie Ganhua , Liu Xubo , Helms Brett A. , Russell Thomas P. 
Published: March 2019

AbstractLiquid–fluid interfaces provide a platform both for structuring liquids into complex shapes and assembling dimensionally confined, functional nanomaterials. Historically, attention in this area has focused on simple emulsions and foams, in which surface‐active materials such as surfactants or colloids stabilize structures against coalescence and alter the mechanical properties of the interface. In recent decades, however, a growing body of work has begun to demonstrate the full potential of the assembly of nanomaterials at liquid–fluid interfaces to generate functionally advanced, biomimetic systems. Here, a broad overview is given, from fundamentals to applications, of the use of liquid–fluid interfaces to generate complex, all‐liquid devices with a myriad of potential applications.
In Situ Synchrotron X‐ray Characterization Shining Light on the Nucleation and Growth Kinetics of Colloidal Nanoparticles
Authors: Wu Siyu , Li Mingrui , Sun Yugang 
Published: March 2019

AbstractRational synthesis of colloidal nanoparticles with desirable properties relies on precise control over the nucleation and growth kinetics, which is still not well understood. The recent development of in situ high energy synchrotron X‐ray techniques offers an excellent opportunity to quantitatively monitor the growth trajectories of colloidal nanoparticles in real time under real reaction conditions. The time‐resolved, quantitative data of the growing colloidal nanoparticles are unique to reveal the mechanism of nanoparticle formation and determine the corresponding intrinsic kinetic parameters. This review discusses the kinetics of major steps of forming colloidal nanoparticles and the capability of in situ synchrotron X‐ray techniques in studying the corresponding kinetics.
Hexanuclear 3d − 4f metal–organic cages assembled from a carboxylic acid‐functionalized tris‐triazamacrocycle for highly selective fluorescent sensing of picric acid
Authors: Tang Qi , Sun Yao , Li Hong‐Yan , Wu Ji‐Qing , Liang Yu‐Ning , Zhang Zhong 
Published: March 2019

Two LnIII ions are sandwiched by dinuclear CoII building blocks derived from a tris‐triazamacrocyclic ligand bearing pendant carboxylic acid functionality, 1,3,5‐tris((4,7‐bis(2‐carboxyethyl)‐1,4,7‐triazacyclonon‐1‐yl)methyl)‐benzene (H6L), giving rising to two nanoscale heterometallic metal–organic cages formulated as [Co4Ln2(LH2.5)2(H2O)4]·(ClO4)6·NO3·nH2O [Ln = Dy, n = 12 (1); Ln = Yb, n = 9 (2)], whose internal cavity accommodates a guest NO3− anion. Their hexanuclear cage‐like architectures are maintained both in solution and solid states as confirmed by mass spectrum as well as X‐ray diffraction experiments. These two cages display ligand‐based fluorescence emissions and therefore both were chosen to be operated as fluorescent chemosensors for the detection of nitroaromatic compounds. Attractively, these metal–organic cages allow highly selective and sensitive detection of picric acid (PA) over other nitroaromatics in solution and suspension, and the fluorescence resonance energy transfer (FRET) between the cage probes and PA is mainly responsible for the remarkable detection efficiency.
Stereoselective β‐Mannosylation with 2,6‐Lactone‐bridged Thiomannosyl Donor by Remote Acyl Group Participation
Authors: Xu Huanfang , Chen Long , Zhang Qi , Feng Yingle , Zu Yujia , Chai Yonghai 
Published: March 2019

AbstractStereoselective β‐mannosylation has been recognized as one of the greatest challenges of carbohydrate chemistry. Herein, we described a practical method for stereoselective construction of β‐mannosides by using a 2,6‐lactone‐bridged thiomannosyl donor through the remote acyl‐group participation as well as the steric effect of O‐4 substituent. The two effects are enabled through the conversion of a regular mannopyranosyl 4C1 conformation into a 2,6‐lactone bridged conformation. The lactone donor could be readily prepared in three steps on a gram scale and the β‐mannosylation proceeded smoothly with high stereoselectivity for primary, secondary and tertiary alcohol acceptors. In addition, this strategy was successfully applied to the synthesis of a naturally occurring trisaccharide.
Nutritional optimization of Arthrospira platensis for starch and Total carbohydrates production
Authors: Lai Yuen Hing , Puspanadan Soopna , Lee Chee Keong 
Published: March 2019

AbstractPresent study aims to optimize the production of starch and total carbohydrates from Arthrospira platensis. Growing concerns toward unprecedented environmental issues associated with plastic pollution has created a tremendous impetus to develop new biomaterials for the production of bioplastic. Starch‐based biopolymers from algae serve as sustainable feedstock for thermoplastic starch production due to their abundant availability and low cost. A. platensis was cultivated in Zarrouk's medium at 32 ± 1°C and exposed to red light with a photoperiod of 12:12 hr light/dark. Growth kinetics studies showed that the maximum specific growth rate (μmax) obtained was 0.059 day−1 with the doubling time (td) of 11.748 days. Subsequently, Zarrouk's medium with different concentrations of sulfur, phosphorus and nitrogen was prepared to establish the nutrient‐limiting conditions to enhance the accumulation of starch and total carbohydrates. In this study, the highest starch accumulated was 6.406 ± 0.622 mg L−1 under optimized phosphorus limitation (0.025 g L−1) conditions. Nitrogen limitation (0.250 g L−1) results demonstrated significant influenced (p < 0.05) on total carbohydrates (67.573 ± 2.893 mg L−1) accumulation in A. platensis. The starch accumulation in A. platensis was significantly affected (p < 0.05) by phosphorus limitation (0.0025 g L−1). Subsequently, the optimized phosphorus concentration was coupled with mixotrophic cultivation to further enhance the starch accumulation. The results obtained indicated that, the starch (11.426 ± 0.314 mg L−1) and carbohydrates (43.053 ± 2.986 mg L−1) concentration obtained was significantly high (p < 0.05) under mixotrophic cultivation. Therefore, it shown that nutrient limitation and mixotrophic cultivation are viable strategies to enhance the accumulation of starch and total carbohydrates in A. platensis.
Efficient Itaconic acid production via protein–protein scaffold introduction between GltA, AcnA, and CadA in recombinant Escherichia coli
Authors: Tran Kim‐Ngan T. , Somasundaram Sivachandran , Eom Gyeong Tae , Hong Soon Ho 
Published: March 2019

AbstractItaconic acid, which is a promising organic acid in synthetic polymers and some base‐material production, has been produced by Aspergillus terreus fermentation at a high cost. The recombinant Escherichia coli that contained the cadA gene from A. terreus can produce itaconic acid but with low yield. By introducing the protein–protein scaffold between citrate synthesis, aconitase, and cis‐aconitase decarboxylase, 5.7 g/L of itaconic acid was produced, which is 3.8‐fold higher than that obtained with the strain without scaffold. The optimum pH and temperature for itaconic acid production were 8.5 and 30°C, respectively. When the competing metabolic network was inactivated by knock‐out mutation, the itaconic acid concentration further increased, to 6.57 g/L.
Utility of the Medtronic microvascular plug™ as a transcatheter implantable and explantable pulmonary artery flow restrictor in a swine model
Authors: Khan Abdul H. , Hoskoppal Deepthi , Kumar T. K. Susheel , Bird Lindsey , Allen Kimberly , Lloyd Hannah , Knott‐Craig Christopher J. , Waller B. Rush , Sathanandam Shyam 
Published: March 2019

AbstractBackgroundA surgical pulmonary artery band (PAB) is used to control excessive pulmonary blood flow for certain congenital heart diseases. Previous attempts have been made to develop a transcatheter, implantable pulmonary flow restrictor (PFR) without great success. We modified a microvascular plug (MVP) to be used as a PFR. The objectives of this study were to demonstrate feasibility of transcatheter implantation and retrieval of the modified MVP as a PFR, and compare PA growth while using the PFR versus PAB.Methods and resultsThe PFR was implanted in eight newborn piglets in bilateral branch pulmonary arteries (PAs). Immediately post‐PFR implantation, the right ventricular systolic pressure increased from a median of 20–51 mmHg. Transcatheter retrieval of PFR was 100% successful at 3, 6, and 9 weeks and 50% at 12‐weeks post‐implant. A left PAB was placed via thoracotomy in four other newborn piglets. Debanding was performed 6‐weeks later via balloon angioplasty. On follow‐up, the proximal left PA diameters in the PFR and the PAB groups were similar (median 8 vs. 7.1 mm; p = 0.11); albeit the surgical band sites required repeat balloon angioplasty secondary to recurrent stenosis. By histopathology, there was grade II vessel injury in two pigs immediately post‐retrieval of PFR that healed by 12 weeks.ConclusionsTranscatheter implantation and retrieval of the MVP as a PFR is feasible. PA growth is comparable to surgical PAB, which is likely to require reinterventions. The use of the MVP as a PFR in humans has to be trialed before recommending its routine use.
Impact of revascularization versus medical therapy alone for chronic total occlusion management in older patients
Authors: Flores‐Umanzor Eduardo Josué , Vázquez Sara , Cepas‐Guillen Pedro , Ivey‐Miranda Juan , Caldentey Guillem , Jimenez‐Britez Gustavo , Regueiro Ander , Freixa Xavier , Andrea Rut , Ferreira‐González Ignacio , Sabaté Manel , Martin‐Yuste Victoria 
Published: March 2019

AbstractBackgroundChronic total occlusions (CTOs) are present in more than one third of older patients with myocardial ischemia, but controversy remains about the best therapeutic approach.AimsTo compare long‐term survival after CTO revascularization (percutaneous coronary intervention [PCI] or coronary artery bypass graft [CABG]) versus medical treatment (MT) alone in patients aged 75 and older.Methods and resultsA total of 1,252 consecutive patients with at least one CTO were identified from 2010 to 2014 in our center. Patients were stratified by age (<75 years vs. ≥75 years) in the present analysis. All‐cause and cardiac mortality were assessed at a median follow‐up of 3.5 years.In the older subgroup (26%), patients were more likely to be treated with MT alone (71% vs. 43% of younger patients; p < 0.001). Patients undergoing revascularization were younger and had higher left ventricular ejection fraction (LVEF) and lower age, creatinine, ejection fraction (ACEF) score (age/LVEF +1 if creatinine >2.0 mg/dL), compared to the MT group (p < 0.05). As compared to MT, revascularization predicted lower rates of cardiac mortality and all‐cause mortality in older patients, both in the subgroups treated with CABG (hazard ratio [HR] 0.35, 95% confidence interval [CI] 0.17–0.71; HR 0.39, 95%CI 0.18–0.81) and PCI (HR 0.57, 95%CI 0.33–0.98; HR 0.59, 95%CI 0.28–1.2). No differences in mortality were observed according to type of revascularization procedure.ConclusionsAmong patients aged at least 75 years with a CTO, revascularization (PCI or CABG) rather than MT alone may portend a better outcome in terms of all‐cause and cardiac mortality.
Ordered Mesoporous CeO2‐supported Ag as an Effective Catalyst for Carboxylative Coupling Reaction Using CO2
Authors: Zhang Xiao , Wang Dingkun , Jing Meizan , Liu Jian , Zhao Zhen , Xu Guanhua , Song Weiyu , Wei Yuechang , Sun Yuanqing 
Published: March 2019

AbstractAg/Mesoporous CeO2 (Ag/M−CeO2) catalysts were synthesized through the gas bubbling‐assisted membrane reduction (GBMR) method and characterized by XRD, ICP‐OES, N2 adsorption‐desorption, XPS, Raman spectroscopy, TEM, HRTEM, HAADF‐STEM and CO2‐TPD. Ag/M−CeO2 catalysts contain uniform mesoporous structure, large surface area and oxygen vacancies, which promote the dispersion of Ag nanoparticles. Density functional theory (DFT) calculations indicate that Ag nanoparticles supported on M−CeO2 could facilitate the formation of oxygen vacancies, which result in higher CO2 adsorption capacity. With the most oxygen vacancies and the well‐dispersed Ag active species, Ag (3.12 %)/M−CeO2 exhibits high efficiency for the carboxylative coupling of terminal alkynes, chloride compounds and CO2 under mild reaction conditions (60 °C, 0.5 MPa), affording a wide range of functionalized 2‐alkynoates in good yields.
Engineered MOFs and Enzymes for the Synthesis of Active Pharmaceutical Ingredients
Authors: Cirujano Francisco G. 
Published: March 2019

AbstractThe latest strategies in the design and synthesis of metal‐organic frameworks as heterogeneous catalysts in organic synthesis are compared with biocatalyzed transformations, as a step forward to a more convenient industrial production of pharmaceuticals. Relevant C−C and C−N bond forming reactions that are both chemo‐ and bio‐ catalyzed by synthetic MOFs and in vitro evolved and/or promiscuous enzymes with the aim of obtaining key bioactive scaffolds in a sustainable, competitive and cost‐efficient manner, are thoroughly discussed in this mini review.