bluuebunny/crossref_metadata_2025_split huggingface dataset:
Systemic and ocular manifestations of a patient with mosaic ARID1A‐associated Coffin‐Siris syndrome and review of select mosaic conditions with ophthalmic manifestations
Authors: Miraldi Utz Virginia , Brightman Diana S. , Sandoval Monica A. , Hufnagel Robert B. , Saal Howard M. 
Published: September 2020

AbstractMosaic genetic mutations may be somatic, germline, or “gonosomal” and have the potential to cause genetic syndromes, disorders, or malformations. Mutations can occur at any point in embryonic development and the timing determines the extent of distribution of the mutation throughout the body and different tissue types. The eye and visual pathway offer a unique opportunity to study somatic and gonosomal mosaic mutations as the eye consists of tissues derived from all three germ layers allowing disease pathology to be assessed with noninvasive imaging. In this review, we describe systemic and ocular manifestations in a child with mosaic Coffin‐Siris syndrome. The patient presented with a significant medical history of accommodative esotropia and hyperopia, macrocephaly, polydactyly, global developmental delay, hypotonia, ureteropelvic junction (UPJ) obstruction, and brain MRI abnormalities. The ophthalmic findings in this patient were nonspecific, however, they are consistent with ocular manifestations reported in other patients with Coffin‐Siris syndrome. We also review ophthalmic findings of select mosaic chromosomal and single‐gene disorders. Ophthalmic assessment alongside clinical genetic testing may play an important role in diagnosis of genetic syndromes as well as understanding disease pathology, particularly when mosaicism plays a role.
The new landscape of retinal gene therapy
Authors: Ku Cristy A. , Pennesi Mark E. 
Published: September 2020

AbstractNovel therapeutics for inherited retinal dystrophies (IRDs) have rapidly evolved since groundbreaking clinical trials for LCA due to RPE65 mutations led to the first FDA‐approved in vivo gene therapy. Since then, advancements in viral vectors have led to more efficient AAV transduction and developed other viral vectors for gene augmentation therapy of large gene targets. Furthermore, significant developments in gene editing and RNA modulation technologies have introduced novel capabilities for treatment of autosomal dominant diseases, intronic mutations, and/or large genes otherwise unable to be treated with current viral vectors. We highlight strategies currently being evaluated in gene therapy clinical trials and promising preclinical developments for IRDs.
Improved behavioral indices of welfare in continuous compared to intermittent pair‐housing in adult female rhesus macaques (Macaca mulatta)
Authors: Cassidy Lauren C. , Hannibal Darcy L. , Semple Stuart , McCowan Brenda 
Published: September 2020

AbstractLimiting opportunities for captive nonhuman primates (NHPs) to express species‐specific social behaviors may disrupt the adaptive drive for social companionship and may lead to increases in coping behaviors and inactivity. While captive NHPs show improved welfare when moving to pair‐housing from single‐housing, the impact of daily separation of pair‐mates, as is implemented in intermittent pair‐housing, is not fully understood. We compared behavioral indices of welfare exhibited by adult female rhesus macaques (Macaca mulatta) in two conditions: (1) intermittent pair‐housing, involving daily overnight separation of pair‐mates, and (2) continuous pair‐housing, involving little separation of pair‐mates. A within‐subjects study design tested two groups of females experiencing both pairing conditions in an alternate order, switching either from continuous to intermittent pair‐housing, or from intermittent to continuous pair‐housing. Behavioral observations, recording activity state, self‐directed, abnormal, and social behaviors, were conducted at midday when all females were paired, and in the afternoon when intermittent pairs were separated. Females exhibited higher levels of inactivity and self‐directed behavior when separated due to intermittent pair‐housing in comparison to continuous pair‐housing. In addition, intermittently paired females showed higher levels of grooming and other types of affiliation when paired, than during the same time frame when they were continuously paired. These results suggest that females in the continuous presence of a social partner experience improved levels of activity and do not need to elevate levels of behavioral coping mechanisms (e.g., self‐scratching, increased affiliation) as they receive the benefits associated with social companionship consistently throughout the day. Overall, this study provides the first evidence that continuous pair‐housing affords better welfare than intermittent pair‐housing in adult female rhesus macaques. Pair‐housing options, such as continuous pairing, that reduce reliance on behavioral coping mechanisms and promote adaptive social behavior throughout the entirety of the day should be prioritized over husbandry care scheduled for convenience.
Hydrogen isotopes in serial hair samples record season of death in a mummified child from 19th century San Francisco, CA
Authors: Eerkens Jelmer W. , Ryder Alex , Evoy Angela , Hull Bryna 
Published: September 2020

AbstractBackgroundThe mummified body of a small child was found in a sealed Barstow cast iron casket during construction activity in San Francisco in 2016. Using historical records and ancient DNA the child was determined to be Edith H. Cook. She was born 28 November, 1873 in the city of San Francisco, and died of “marasmus” on 13 October, 1876 also in San Francisco.AimsCurrently, there are few techniques for estimating human season of death in archaeological cases. Hydrogen isotope ratios (δ2H) in hair keratin is known to strongly correlate with drinking water. We explore δ2H in serial hair samples as a potential technique to estimate season of death by comparing the δ2H record from hair to the known date of death.Materials & MethodsApproximately 50 hairs were removed from the scalp, aligned from the root, and cut into 5cm serial sections, each representing approximately 2 weeks of growth, and the total sequence a total of 1 year of growth. δ2H was measured on each 5cm segment and compared to previously‐reported δ13C, δ15N, and δ34S values.Resultsδ2H in the serial hair samples ranged between −56‰ and −48‰, consistent with her water values recorded in surface waters from San Francisco, and follow a sinusoidal pattern. Decreasing δ2H in terminal samples before death suggest Fall as the season of death, consistent with the known date of death.DiscussionThis test case shows that archaeological hair preserves a seasonal signature in the form of changing keratin δ2H values that correlate to changing δ2H in surface drinking water. Terminal values in root record water ingested during the final week(s) before death.ConclusionsWe argue that this technique can be used to estimate season of death in future archaeological or forensic cases where hair has been preserved but date of death is unknown.
Sonographic ‘flash’: A case report of a new sign in dynamic ultrasound of extra‐articular snapping hip and knee
Authors: Koh Eamon 
Published: September 2020

AbstractSnapping phenomena at the hip and knee are common clinical presentations in the general population and athletes. Dynamic ultrasound is a useful tool in the evaluation of these syndromes, especially when intra‐articular pathology has been excluded. We report on a new ultrasound sign, the ‘sonographic flash’, which identified the source of audible snapping in two cases where snapping phenomena were present.
Reaction Pathways and Redox States in α‐Selective Cobalt‐Catalyzed Hydroborations of Alkynes
Authors: Blasius Clemens K. , Vasilenko Vladislav , Matveeva Regina , Wadepohl Hubert , Gade Lutz H. 
Published: September 2020

AbstractCobalt(II) alkyl complexes supported by a monoanionic NNN pincer ligand are pre‐catalysts for the regioselective hydroboration of terminal alkynes, yielding the Markovnikov products with α:β‐(E) ratios of up to 97:3. A cobalt(II) hydride and a cobalt(II) vinyl complex appear to determine the main reaction pathway. In a background reaction the highly reactive hydrido species specifically converts to a coordinatively unsaturated cobalt(I) complex which was found to re‐enter the main catalytic cycle.
Switching the Photoluminescence and Electrochemiluminescence of Liposoluble Porphyrin in Aqueous Phase by Molecular Regulation
Authors: Zhang Yinpan , Zhao Yaqi , Han Zhengang , Zhang Ruizhong , Du Peiyao , Wu Yanxia , Lu Xiaoquan 
Published: September 2020

AbstractBy a facile peripheral decoration of 5‐(4‐aminophenyl)‐10,15,20‐triphenylporphyrin (ATPP) with inherent aggregation‐induced emission (AIE) active tetraphenylethene (TPE), a versatile AIEgenic porphyrin derivative (ATPP‐TPE) was obtained, which greatly abolishes the detrimental π–π stacking and thus surmounts the notorious aggregation‐caused quenching (ACQ) effect of ATPP in aqueous phase. The photoluminescence of ATPP‐TPE is 4.5‐fold stronger than ATPP at aggregation state. Moreover, an unequivocal aggregation induced electrochemiluminescence (AIECL) of ATPP‐TPE was found to be seriously dependent on its aggregation property in aqueous solution with efficiency of 34 %, which is 6 times higher than pure ATPP. The versatility of this molecular structure modulation strategy along with the ACQ‐to‐AIE transformation in this work provides direction to guide for applying liposoluble porphyrins in aqueous phase by designs of synthetic porphyrin AIEgens.
An Ester‐Substituted Semiconducting Polymer with Efficient Nonradiative Decay Enhances NIR‐II Photoacoustic Performance for Monitoring of Tumor Growth
Authors: Zha Menglei , Lin Xiangwei , Ni Jen‐Shyang , Li Yaxi , Zhang Yachao , Zhang Xun , Wang Lidai , Li Kai 
Published: September 2020

AbstractPhotoacoustic agents have been of vital importance for improving the imaging contrast and reliability against self‐interference from endogenous substances. Herein, we synthesized a series of thiadiazoloquinoxaline (TQ)‐based semiconducting polymers (SPs) with a broad absorption covering from NIR‐I to NIR‐II regions. Among them, the excited s‐BDT‐TQE, a repeating unit of SPs, shows a large dihedral angle and narrow adiabatic energy as well as low radiative decay, attributing to its strongly electron‐deficient ester‐substituted TQ‐segment. In addition, its more vigorous molecular motions trigger a higher reorganization energy that further yields an efficient photoinduced nonradiative decay, which has been carefully examined and understood by theoretical calculation. Thus, BDT‐TQE SP‐cored nanoparticles with twisted intramolecular charge transfer (TICT) feature exhibit a high NIR‐II photothermal conversion efficiency (61.6 %) and preferable PA tracking of in situ hepatic tumor growth for more than 20 days. This study highlights a unique strategy for constructing efficient NIR‐II photoacoustic agents via TICT‐enhanced PNRD effect, advancing their applications for in vivo bioimaging.
Ultrastable Perovskite–Zeolite Composite Enabled by Encapsulation and In Situ Passivation
Authors: Wang Peijun , Wang Bolun , Liu Yucheng , Li Lin , Zhao Hua , Chen Yonghua , Li Jiyang , Liu Shengzhong (Frank) , Zhao Kui 
Published: September 2020

AbstractMetal halide perovskites have been widely applied in optoelectronic fields, but their poor stability hinders their actual applications. A perovskite–zeolite composite was synthesized via in situ growth in air from aluminophosphate AlPO‐5 zeolite crystals and perovskite nanocrystals. The zeolite matrix provides quantum confinement for perovskite nanocrystals, achieving efficient green emission, and it passivates the defects of perovskite by H‐bonding interaction, which leads to a longer lifetime compared to bulk perovskite film. Furthermore, the AlPO‐5 zeolite also acts as a protection shield and enables ultrahigh stability of perovskite nanocrystals under 150 °C heat stress, under a 15‐month long‐term ambient exposure, and even in water for more than 2 weeks, respectively. The strategy of in situ passivation and encapsulation for the perovskite@AlPO‐5 composite was amenable to a range of perovskites, from MA‐ to Cs‐based perovskites. Benefiting from high stability and photoluminescence performance, the composite exhibits great potential to be virtually applied in light‐emitting diodes (LEDs) and backlight displays.
Enantioselective Total Synthesis of (−)‐Finerenone Using Asymmetric Transfer Hydrogenation
Authors: Lerchen Andreas , Gandhamsetty Narasimhulu , Farrar Elliot H. E. , Winter Nils , Platzek Johannes , Grayson Matthew N. , Aggarwal Varinder K. 
Published: September 2020

Abstract(−)‐Finerenone is a nonsteroidal mineralocorticoid receptor antagonist currently in phase III clinical trials for the treatment of chronic kidney disease in type 2 diabetes. It contains an unusual dihydronaphthyridine core. We report a 6‐step synthesis of (−)‐finerenone, which features an enantioselective partial transfer hydrogenation of a naphthyridine using a chiral phosphoric acid catalyst with a Hantzsch ester. The process is complicated by the fact that the naphthyridine exists as a mixture of two atropisomers that react at different rates and with different selectivities. The intrinsic kinetic resolution was converted into a kinetic dynamic resolution at elevated temperature, which enabled us to obtain (−)‐finerenone in both high yield and high enantioselectivity. DFT calculations have revealed the origin of selectivity.
The Optical Spectrum of Au2+
Authors: Förstel Marko , Pollow Kai Mario , Saroukh Karim , Najib Este Ainun , Mitric Roland , Dopfer Otto 
Published: September 2020

AbstractThe electronic structure of the Au2+ cation is essential for understanding its catalytic activity. We present the optical spectrum of mass‐selected Au2+ measured via photodissociation spectroscopy. Two vibrationally resolved band systems are observed in the 290–450 nm range (at ca. 440 and ca. 325 nm), which both exhibit rather irregular structure indicative of strong vibronic and spin‐orbit coupling. The experimental spectra are compared to high‐level quantum‐chemical calculations at the CASSCF‐MRCI level including spin‐orbit coupling. The results demonstrate that the understanding of the electronic structure of this simple, seemingly H2+‐like diatomic molecular ion strictly requires multireference and relativistic treatment including spin‐orbit effects. The calculations reveal that multiple electronic states contribute to each respective band system. It is shown that popular DFT methods completely fail to describe the complex vibronic pattern of this fundamental diatomic cation.
Characterizing Lecithin Interactions in Chocolate Using Interfacial Properties and Rheology
Authors: Caparosa Monica H. , Hartel Richard W. 
Published: September 2020

AbstractThe functionality of chocolate is based on specific properties including how it flows. Cocoa butter and lecithin are two ingredients that help to control the flow properties of melted chocolate. Lecithin is an emulsifier that has been the preferred method of providing yield stress and viscosity control while reducing production costs by decreasing the amount of cocoa butter needed to achieve similar flow results. In this industrially oriented study, soy, sunflower, and dairy lecithin were compared by quantifying interfacial and rheological properties of lecithin in dark chocolate. Each lecithin source was added up to the 1.0% limit, which is the emulsifier maximum set forth by the Code of Federal Regulations. The compositions of each lecithin source were analyzed for total phospholipid composition, which were used to normalize data based on the total added phosphatides added for all samples. Differences in both phosphatide composition and fatty acid composition were found. Both interfacial tension between oil and water phases and contact angle between a cocoa butter drop and flat sucrose crystal surface decreased as the phosphatide concentration increased for each lecithin source. In terms of rheology, the yield stress first decreased with increasing lecithin content and then began to increase, whereas plastic viscosity continually decreased with addition of lecithin. Significant differences were seen for the different lecithin sources, even when compared on an equivalent phosphatide content. No correlations were found between interfacial properties and yield stress; however, direct correlations were found between both interfacial measures and plastic viscosity.
Fabrication and characterization of poly(1,4‐cyclohexanedimthylene terephthalate‐co‐1,4‐cyclohxylenedimethylene 2,6‐naphthalenedicarboxylate) (PCTN) copolyester film: A novel copolyester film with exceptional performances for next generation flexible electronic devices
Authors: Hussain Fiaz , Park Sangwon , Jeong Jaemin , Jeong Euichul , Kang Soo‐Jung , Yoon Kyunghwan , Kim Jinhwan 
Published: September 2020

AbstractA novel, semi‐crystalline poly(1,4‐cyclohexylenedimethylene terephthalate‐co‐1,4‐cyclohexylene dimethylene 2,6‐naphthalenedicarboxylate) (PCTN) copolyester was synthesized and successfully extruded using an extruder attached with T‐die. The extruded sheet was uniaxially cold‐drawn into film at 150°C, a temperature between glass transition temperature and melting temperature. The mechanical, thermal, thermo‐mechanical, water barrier, and optical properties of developed film were characterized. Orientation of different phases was analyzed by X‐ray diffraction. The actual chemical composition of PCTN copolyester was analyzed by nuclear magnetic resonance spectroscopy. Our results clearly indicated the thermal, dimensional stability, mechanical, and water barrier properties of PCTN films increased in a linear trend with draw ratio. It was also found that PCTN film has better water barrier property compared to conventional poly(ethylene terephthalate) (PET) and poly(ethylene 2,6‐naphthalate) (PEN) films. Based on the exceptional performance properties, novel PCTN film may find a strong position as a substrate for next generation flexible electronics among various performance materials.
Correlation between the mechanical and dielectric responses in polymer films by a fractional calculus approach
Authors: Rentería‐Baltiérrez Flor Yanhira , Reyes‐Melo Martín Edgar , Puente‐Córdova Jesús Gabino , López‐Walle Beatriz 
Published: September 2020

AbstractA fractional calculus approach was used to study the correlation between the complex elastic modulus and the complex relative permittivity for a polystyrene (PS) film with thickness of ~80 μm. Experimental measurements were carried out using dynamic mechanical analysis and dynamic dielectric analysis. Experimental results show the mechanical and dielectric manifestations of the main relaxation (glass transition process), whose molecular mobility was analyzed by two innovative models: a mechanical fractional model and a dielectric fractional model. Parameters of fractional models show that, when temperature increases, the molecular mobility of the main relaxation also increases, but the cooperativity of mobility decreases. Besides, molecular mobility is greater in the mechanical manifestation of the main relaxation than in the electric manifestation. From theoretical results obtained from fractional models for the isochronal mechanic storage modulus, E′(T), and the isochronal relative permittivity, , a correlation model for mechanical and dielectric properties of PS film was obtained. This correlation model describes in function of E′(T). These results suggest that this correlation model can be used to study molecular mobility of mechanical and dielectric dynamic properties of the polymer films samples and predict changes in their behavior by modifying ambient conditions.
Geomorphological and non‐destructive GPR survey for the conservation of frescos in the rupestrian churches of Matera (Basilicata, southern Italy)
Authors: De Giorgi Lara , Lazzari Maurizio , Leucci Giovanni , Persico Raffaele 
Published: September 2020

AbstractIn this article we present the results of a geomorphological and geophysical investigation carried out in the Regional Park of Matera Murgia, also known as Park of the Rupestrian Churches (world site protected by UNESCO), in Basilicata region, southern Italy. The aim of this investigation is to support the scheduled restoration works in some rupestrian historical sites and the safety of the tourist route that connects the churches along the eastern side of Gravina river valley. In particular, a ground penetrating radar (GPR) prospection was conducted on frescoed surfaces in two rupestrian churches permitting the detection of different kinds of problems affecting the fresco surfaces, such as lesions and efflorescence.
Cover Image, Volume 14, Issue 5
Authors: Zemanek Debrah , Champagne Pascale , Mabee Warren 
Published: September 2020

The front cover image © David Blair is based on the Review Article Review of Life Cycle Greenhouse Gas Emissions Assessments of Hydroprocessed Renewable Fuel (HEFA) from Oilseeds by Debrah Zemanek et al., https://doi.org/10.1002/bbb.2125.
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Prognosis and safety of radium‐223 with concurrent abiraterone acetate or enzalutamide use for metastatic castration‐resistant prostate cancer: Real‐world data of Japanese patients
Authors: Miyoshi Yasuhide , Yasui Masato , Ttsutsumi Sohgo , Kawahara Takashi , Uemura Ko‐ichi , Hayashi Naruhiko , Nozawa Masahiro , Yoshimura Kazuhiro , Uemura Hiroji , Uemura Hirotsugu 
Published: September 2020

AbstractObjectivesTo evaluate the real‐world data on the efficacy and safety of a combination therapy with radium‐223 (Ra‐223) and second‐generation androgen‐receptor targeting agents (ARTAs), including abiraterone acetate (ABI) and enzalutamide (ENZ), among Japanese patients with bone metastatic castration‐resistant prostate cancer (CRPC).Patients and methodsWe retrospectively reviewed 79 patients with bone metastatic CRPC who were treated with Ra‐223. The number of patients with concurrent ARTA use was 24:17 receiving ABI and 7 receiving ENZ. We evaluated the overall survival (OS) according to ARTA use and compared the survival of patients treated with Ra‐223 with or without ARTA using multivariate analysis.ResultsThe median survival in the entire cohort was 23.5 months. The patients receiving Ra‐223 combined with ARTA showed a tendency of better OS than patients treated with Ra‐223 alone, although no significant difference was observed (median OS, 26.5 vs 23.5 months; P = .115). A multivariate analysis showed that the extent of disease on bone scan (EOD) scores and pain at baseline were significant predictors of OS. The concurrent use of bone‐modifying agents (BMAs) was not significant for favorable OS (P = .050). However, the concurrent use of second‐generation ARTA was not a significant factor for OS. Regarding safety, a bone fracture occurred in only one (4.2%) of 24 patients treated with combined Ra‐223 and ARTA therapy.ConclusionOur real‐world data analysis suggested that Ra‐223 combined with a second‐generation ARTA is well tolerated in Japanese patients. The EOD score and pain at baseline are significant prognostic factors for OS, but the concurrent use of second‐generation ARTA has no influence on OS among men treated with Ra‐223. The concurrent use of BMA yields a marginally favorable OS.