bluuebunny/crossref_metadata_2025_split huggingface dataset:
Atypical presentation of ACCES syndrome resembling dominant Spondyloepiphyseal dysplasia tarda
Authors: Sezer Abdullah , Özdemir Zeynep , Özkan Erdem , Çetinkaya Semra 
Published: August 2024

AbstractAplasia Cutis Congenita with Ectrodactyly Skeletal Syndrome (ACCES, OMIM #619959) is an extremely rare multiple congenital anomalies syndrome caused by haploinsufficiency of the UBA2 gene. This syndrome presents with growth retardation, dysmorphic facial features, neurodevelopmental delay, skeletal problems including ectrodactyly, developmental dysplasia of the hip (DDH) and scoliosis, skin findings such as aplasia cutis, and some internal organ abnormalities. Our 13‐year‐old female patient and her 38‐year‐old father had a skeletal dysplasia phenotype with disproportionate short stature, bilateral DDH, mild epiphyseal involvement, scoliosis, and increased lumbar lordosis. Both were neurodevelopmentally normal and had mild dysmorphic facial features and mild ectodermal findings. The dominant inheritance pattern in the pedigree suggested a pre‐diagnosis of spondyloepiphyseal dysplasia tarda. The exome sequencing analysis of the patient has identified a novel heterozygous variant, NM_005499.2:c.460‐2A >G, in the UBA2 gene, and the father was found heterozygous either. The isolated spondyloepiphyseal involvement of our patients was an unusual presentation compared to patients with ACCES syndrome previously reported in the literature. Considering the highly variable expressiveness of ACCES syndrome and the co‐occurrence of familial hip dysplasia and vertebral problems, we suggest that this syndrome can also be classified under “Spondyloepi(meta)physial dysplasia (SE(M)D)” in the nosology of genetic skeletal disorders.
The DNH 7 endocast of Paranthropus robustus from Drimolen, South Africa: Reconsidering the functional significance of an enlarged occipital‐marginal (O/M) sinus system in robust australopithecines
Authors: Falk Dean , Marom Assaf 
Published: August 2024

AbstractThis paper presents a detailed analysis of the endocast of one of the most complete Paranthropus robustus crania known, DNH 7, from the Drimolen site (South Africa), and compares it with the morphology of other australopithecine endocasts. We focus on endocranial volume, the impressions of cortical sulci, cranial sutures, and the pattern of cranial venous sinuses on the endocast. A noteworthy observation is the estimated endocranial capacity of 403 cm3, which is small for an adult Paranthropus. Fragmentary sulci identified in the frontal and temporal lobes of DNH 7 exhibit similarities with patterns observed in chimpanzees and gracile australopithecines. We observe the presence of a large remnant of an occipital‐marginal sinus on DNH 7 and provide an updated table of 13 Paranthropus endocasts that are scorable for this trait, which reinforces the hypothesis that an enlarged occipital‐marginal (O/M) sinus system was fixed across the three species of Paranthropus. In light of this, the possible functional significance of the occipital‐marginal sinus system is reevaluated considering the ontogenetic development of cranial venous blood flow in human children. This leads us to hypothesize that the ontogenetic development of cranial blood flow in Paranthropus and Australopithecus africanus infants were different and to suggest that Taung 1 was the only A. africanus specimen known to have exhibited an enlarged O/M sinus system because it was an immature individual.
One‐Stone‐For‐Three‐Birds Strategy Using a Fullerene Modifier for Efficient and Stable Inverted Perovskite Solar Cells
Authors: Jiang Xiaofen , Jia Lingbo , Zhang Shantao , Gao Yan , Yan Nan , Hou Tianao , Gao Shuang , Wang Xue , Li Xinyu , Chen Wenjing , Xiao Zhengguo , Wu Xiaojun , Fang Zhimin , Liu Shengzhong (Frank) , Yang Shangfeng 
Published: August 2024

AbstractThe electron extraction from perovskite/C60 interface plays a crucial role in influencing the photovoltaic performance of inverted perovskite solar cells (PSCs). Here, we develop a one‐stone‐for‐three‐birds strategy via employing a novel fullerene derivative bearing triple methyl acrylate groups (denoted as C60‐TMA) as a multifunctional interfacial layer to optimize electron extraction at the perovskite/C60 interface. It is found that the C60‐TMA not only passivates surface defects of perovskite via coordination interactions between C=O groups and Pb2+ cations but also bridge electron transfer between perovskite and C60. Moreover, it effectively induces the secondary grain growth of the perovskite film through strong bonding effect, and this phenomenon has never been observed in prior art reports on fullerene related studies. The combination of the above three upgrades enables improved perovskite film quality with increased grain size and enhanced crystallinity. With these advantages, C60‐TMA treated PSC devices exhibit a much higher power conversion efficiency (PCE) of 24.89 % than the control devices (23.66 %). Besides, C60‐TMA benefits improved thermal stability of PSC devices, retaining over 90 % of its initial efficiency after aging at 85 °C for 1200 h, primarily due to the reinforced interfacial interactions and improved perovskite film quality.
Molecular Design of Hole Transport Materials to Immobilize Ion Motion for Photostable Perovskite Solar Cells
Authors: Zhang Zheng , Duan Chenghao , Wang Sijing , Xie Tianyou , Zou Feilin , Luo Yang , Tang Ruijia , Guo Kunpeng , Yuan Ligang , Zhang Kaicheng , Wang Yao , Qiu Jianhang , Yan Keyou 
Published: August 2024

AbstractPoor operational stability is a crucial factor limiting the further application of perovskite solar cells (PSCs). Organic semiconductor layers can be a powerful means for reinforcing interfaces and inhibiting ion migration. Herein, two hole‐transporting molecules, pDPA‐SFX and mDPA‐SFX, are synthesized with tuned substituent connection sites. The meta‐substituted mDPA‐SFX results in a larger dipole moment, more ordered packing, and better charge mobility than pDPA‐SFX, accompanying with strong interface bonding on perovskite surfaces and suppressed ion motion as well. Importantly, mDPA‐SFX‐based PSCs exhibit an efficiency that has significantly increased from 22.5 % to 24.8 % and a module‐based efficiency of 19.26 % with an active area of 12.95 cm2. The corresponding cell retain 94.8 % of its initial efficiency at maximum power point tracking (MPPT) after 1,000 h (T95=1,000 h). The MPPT T80 lifetime is as long as 2,238 h. This work illustrates that a small degree of structural variation in organic compounds leaves considerable room for developing new HTMs for light stable PSCs.
Three‐Dimensional Covalent Organic Frameworks Based on Linear and Trigonal Linkers for High‐Performance H2O2 Photosynthesis
Authors: Zhu Ruo‐Meng , Liu Yong , Han Wang‐Kang , Feng Jing‐Dong , Zhang Jinfang , Pang Huan , Zhang Jiangwei , Gu Zhi‐Guo 
Published: August 2024

AbstractThe design of three‐dimensional covalent organic frameworks (3D COFs) using linear and trigonal linkers remains challenging due to the difficulty in achieving a specific non‐planar spatial arrangement with low‐connectivity building units. Here, we report the novel 3D COFs with linear and trigonal linkers, termed TMB‐COFs, exhibiting srs topology. The steric hindrance provides an additional force to alter the torsion angles of peripheral triangular units, guiding the linear unit to connect with the trigonal unit into 3D srs frameworks, rather than the more commonly observed two‐dimensional (2D) hcb structures. Furthermore, we comprehensively examined the hydrogen peroxide photocatalytic production capacity of the TMB‐COFs in comparison with analogous 2D COFs. The experimental results and DFT calculations demonstrate a significant enhancement in photocatalytic hydrogen peroxide production efficacy through framework regulation. This work emphasizes the steric configuration using low connectivity building units, offering a fresh perspective on the design and application of 3D COFs.
[4+1] Heteroannulation to Form 1‐Pyrrolines through Alder‐Ene Reaction of in situ Generated Ynimines: Development and Application in Total Synthesis of Borrecapine
Authors: Lavernhe Rémi , Wang Qian , Zhu Jieping 
Published: August 2024

AbstractWe report in this paper a novel Cu‐catalyzed synthesis of polysubstituted 1‐pyrrolines. The reaction of β,γ‐unsaturated oxime esters 4 with terminal alkynes 5 in the presence of a catalytic amount of Cu(OAc)2 and 2,2′‐biquinoline affords the corresponding 1,6‐enynimines, which undergo a highly stereoselective Alder‐ene reaction to afford 1‐pyrrolines with concomitant generation of a quaternary carbon and a 2‐azadiene motif. It represents an unusual [4+1] heteroannulation reaction wherein terminal alkynes act as a one carbon donor and are 1,1‐difunctionalized. Mechanistic studies suggest that the allylic hydrogen trans to the oxime ester main chain is selectively transferred to the electron‐rich alkyne during the pericyclic reaction. The resulting 1‐pyrrolines undergo facile acid‐catalyzed regioselective Wagner–Meerwein rearrangement to provide 2H‐pyrroles in excellent yields. The 2H‐pyrroles are also accessible from 4 and 5 in a one‐pot manner without isolation of the 1‐pyrrolines. Leveraging this heteroannulation reaction as a key step, the first total synthesis of borrecapine, a 2,3,3,5‐tetrasubstituted pyrrolidine, is accomplished.
Synthesis, in vitro and in silico evaluation of gallamide and selenogallamide derivatives as inhibitors of the SARS‐CoV‐2 main protease
Authors: Carvalho Maryelle A. G. de , Souza Gabriella B. , Tizziani Tiago , Pontes Carime L. M. , Dambrós Bibiana P. , Sousa Natália F. de , Scotti Marcus T. , Steindel Mario , Braga Antonio L. , Sandjo Louis P. , Assis Francisco F. de 
Published: August 2024

AbstractThe present work reports the inhibitory effect of amides derived from gallic acid (gallamides) against severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) main protease (Mpro), along with cytotoxicity evaluation and molecular docking studies. In addition to gallamides, other relevant compounds were also synthesized and evaluated against Mpro, making a total of 25 compounds. Eight compounds presented solubility issues during the inhibitory assay and one showed no inhibitory activity. Compounds 3a, 3b, and 3f showed the highest enzymatic inhibition with IC50 = 0.26 ± 0.19 µM, 0.80 ± 0.38 µM, and 2.87 ± 1.17 µM, respectively. Selenogallamide 6a exhibited IC50 values of 5.42 ± 2.89 µM and a comparison with its nonselenylated congener 3c shows that the insertion of the chalcogen moiety improved the inhibitory capacity of the compound by approximately 10 times. Regarding the cellular toxicity in THP‐1 and Vero cells, compounds 3e and 3g, showed moderate cytotoxicity in Vero cells, while for THP‐1 both were nontoxic, with CC50 > 150 µM. Derivative 3d showed moderate cytotoxicity against both cell lines, whereas 6d was moderatly toxic to THP‐1. Other compounds analyzed do not induce substantial cellular toxicity at the concentrations tested. The molecular docking results for compounds 3a, 3b, and 3f show that hydrogen bonding interactions involving the hydroxyl groups (OH) of the gallate moiety are relevant, as well as the carbonyl group.
Quality of reporting health behaviors for multiple sclerosis (QuoRH‐MS): A scoping review to inform intervention planning and improve consistency of reporting
Authors: Probst Yasmine , Kinnane Emily 
Published: August 2024

AbstractBackgroundMultiple sclerosis (MS) is a neurological condition that necessitates a multidisciplinary approach to aid those living with MS in managing their disease. Health behavior, or lifestyle modification, is an emerging approach to MS self‐management. MS researchers utilize measurement tools to ensure that interventions are best suited to the outcomes, thereby potentially influencing practice. The aim of this study was to investigate which tools are being used for health behavior management studies in people living with MS and develop an aid for tool selection.MethodsA scoping review guided by the PRISMA‐Sc checklist and the JBI manual for evidence synthesis was employed with a systematic search strategy executed across four scientific databases: Medline, PubMed, CINAHL, and Cochrane Libraries. The types of assessment tools used were extracted from the included studies. Each tool was categorized into the health behavior intervention discipline (nutrition, exercise, and psychology) and then subcategorized by the tool's purpose. The frequency of use was determined for each tool. Reporting of validation of the assessment tools were collated to inform a tool selection checklist.ResultsThe review identified a total of 248 tools (12 nutrition, 55 exercise, and 119 psychology unique reports) from 166 studies. Seventy‐seven multidimensional tools were identified including measures of quality of life, fatigue, and functional scales. Only 88 studies (53%) referred to the validity of the tools. The most commonly reported tools were the dietary habits questionnaire (n = 4, nutrition), 6‐minute walk test (n = 17, exercise), Symbol Digits and Modalities Test, and Hospital Anxiety and Depression Scale (n = 15 each, psychology) with the Expanded Disability Status Scale reported 43 times.ConclusionEvidence from interventions may inform practice for health professionals. This review provides insights into the range of tools reported across health behavior intervention studies for MS and offers a guide toward more consistent reporting of study methods.
Factors affecting consumer purchases of natural foods: Prioritizing health consciousness and environmental sustainability
Authors: Pahari Subhajit , Chakraborty Debarun , Polisetty Aruna , Dash Ganesh , Camilleri Mark Anthony , Zhang Justin 
Published: August 2024

AbstractTo date, there are still limited studies that have devoted their attention to the customers' demand for natural food products. Notwithstanding, to the best of the authors' knowledge, there are no articles that integrate the theory of consumption values with the stimuli‐organism‐behavior‐consequence framework, to explore the intricate connections between consumption values, purchase intentions, and actual buying behaviors of natural food products. Primary data were gathered from 793 survey respondents, and a covariance‐based structural equations modeling approach was used to evaluate the robustness of the proposed model. The findings indicate that the various benefits of natural food products have a significant impact on how consumers perceive and approach these products. The results also suggest that consumer behaviors are greatly influenced by their environmental concerns and health considerations. Furthermore, they underline the significance of the consumers' incomes and education levels on the causal paths between attitudes, intentions to purchase, and actual purchase behaviors. This contribution advances a novel theoretical model that is empirically tested, as well as reasonable implications for industry practitioners.
Consumers' purchase behavior of Cradle to Cradle Certified® products—The role of trust and supply chain transparency
Authors: Damberg Svenja , Saari Ulla A. , Fritz Morgane , Dlugoborskyte Vytaute , Božič Katerina 
Published: August 2024

AbstractConsumers' trust in eco‐labels is declining due to the growing number of different kinds of eco‐labels that can be uncertified and related to greenwashing. This paper argues that providing more transparency regarding green supply chains (GSCs) through eco‐labels (such as Cradle to Cradle Certified®) is critical for creating trust and convincing consumers to buy eco‐products over conventional ones. Building on previous literature related to sustainable consumption behavior, green purchasing behavior, Cradle to Cradle certification and GSC management, we develop and test a conceptual model to empirically investigate the links between consumers' trust in eco‐product labels and GSC perceptions in influencing green purchase behavior (GPB) in the case of eco‐friendly fast‐moving consumer goods (FMCGs). By studying a sample of 276 German consumers and applying structural equation modeling, we find that the trust in eco‐product labels and positive perceptions of GSCs are important drivers of GPB. Our research contributes by expanding the knowledge on the factors influencing the acceptance of eco‐products, highlighting the importance of supply chain transparency and trust in GSCs among consumers. We discuss theoretical implications for green product innovation and marketing including eco‐product labeling based on transparent GSCs.
Promising first‐line immuno‐combination therapies for unresectable hepatocellular carcinoma: A cost‐effectiveness analysis
Authors: Wen Feng , Huang Peng , Wu Qiuji , Yang Yang , Zhou Kexun , Zhang Mengxi , Li Qiu 
Published: August 2024

AbstractBackground and AimsHepatocellular carcinoma (HCC) is one of the leading causes of cancer‐related death all over the world, and brings a heavy social economic burden especially in China. Several immuno‐combination therapies have shown promising efficacy in the first‐line treatment of unresectable HCC and are widely used in clinical practice. Nevertheless, which combination is the most affordable one is unknown. Our study assessed the cost‐effectiveness of the immuno‐combinations as first‐line treatment for patients with unresectable HCC from the perspective of Chinese payers.MethodsA Markov model was built according to five multicenter, phase III, open‐label, randomized trials (Himalaya, IMbrave150, ORIENT‐32, CARES‐310, LEAP‐002) to investigate the cost‐effectiveness of tremelimumab plus durvalumab (STRIDE), atezolizumab plus bevacizumab (A + B), sintilimab plus bevacizumab biosimilar (IBI305) (S + B), camrelizumab plus rivoceranib (C + R), and pembrolizumab plus lenvatinib (P + L). Three disease states were included: progression free survival (PFS), progressive disease (PD) as well as death. Medical costs were searched from West China Hospital, published literatures or the Red Book. Cost‐effectiveness ratios (CERs) and incremental cost‐effectiveness ratios (ICERs) were evaluated to compare costs among different combinations. Sensitivity analyses were performed to assess the robust of the model.ResultsThe total cost and quality‐adjusted life years (QALYs) of C + R, S + B, P + L, A + B and STRIDE were $12,109.27 and 0.91, $26,961.60 and 1.12, $55,382.53 and 0.83, $70,985.06 and 0.90, $84,589.01 and 0.73, respectively, resulting in the most cost‐effective strategy of C + R with CER of $13,306.89 per QALY followed by S + B with CER of $24,072.86 per QALY. Compared with C + R, the ICER of S + B strategy was $70,725.38 per QALY, which would become the most cost‐effective when the willing‐to‐pay threshold exceeded $73,500/QALY. In the subgroup analysis, with the application of Asia results in Leap‐002 trial, the model results were the same as global data. In the sensitivity analysis, with the variation of parameters, the results were robust.ConclusionAs one of the promising immuno‐combination therapies in the first‐line systemic treatment of HCC, camrelizumab plus rivoceranib demonstrated the potential to be the most cost‐effective strategy, which warranted further studies to best inform the real‐world clinical practices.
The miRNAs 203a/210‐3p/5001‐5p regulate the androgen/androgen receptor/YAP‐induced migration in prostate cancer cells
Authors: Huo Chieh , Kuo Ying‐Yu , Lin Ching‐Yu , Shiah Shine‐Gwo , Li Chia‐Yang , Huang Shu‐Pin , Chen Jen‐Kun , Wang Wen‐Ching , Kung Hsing‐Jien , Chuu Chih‐Pin 
Published: August 2024

AbstractBackgroundProstate cancer (PCa) patients with elevated level of androgen receptor (AR) correlate with higher metastatic incidence. Protein expression of AR and its target gene prostate‐specific antigen (PSA) are elevated in metastatic prostate tumors as compared to organ‐confined tumors. Androgen treatment or elevation of AR promotes metastasis of PCa in cell culture and murine model. However, under androgen depleted condition, AR suppressed cell mobility and invasiveness of PCa cells. Androgen deprivation therapy in PCa patients is associated with higher risk of cancer metastasis. We therefore investigated the dual roles of AR and miRNAs on PCa metastasis.MethodsThe PC‐3AR (PC‐3 cells re‐expressing AR) and LNCaP cells were used as PCa cell model. Transwell migration and invasion assay, wound‐healing assay, zebrafish xenotransplantation assay, and zebrafish vascular exit assay were used to investigate the role of AR and androgen on PCa metastasis. Micro‐Western Array, co‐immunoprecipitation and Immunofluorescence were applied to dissect the molecular mechanism lying underneath. The miRNA array, miRNA inhibitors or plasmid, and chromatin immunoprecipitation assay were used to study the role of miRNAs on PCa metastasis.ResultsIn the absence of androgen, AR repressed the migration and invasion of PCa cells. When androgen was present, AR stimulated the migration and invasion of PCa cells both in vitro and in zebrafish xenotransplantation model. Androgen increased phospho‐AR Ser81 and yes‐associated protein 1 (YAP), decreased phospho‐YAP Ser217, and altered epithelial‐mesenchymal transition (EMT) proteins in PCa cells. Co‐IP assay demonstrated that androgen augmented the interaction between YAP and AR in nucleus. Knockdown of YAP or treatment with YAP inhibitor abolished the androgen‐induced migration and invasion of PCa cells, while overexpression of YAP showed opposite effects. The miRNA array revealed that androgen decreased hsa‐miR‐5001‐5p but increased hsa‐miR‐203a and hsa‐miR‐210‐3p in PC‐3AR cells but not PC‐3 cells. Treatment with inhibitors targeting hsa‐miR‐203a/hsa‐miR‐210‐3p, or overexpression of hsa‐miR‐5001‐5p decreased YAP expression as well as suppressed the androgen‐induced migration and invasion of PCa cells. Chromatin immunoprecipitation (ChIP) assay demonstrated that AR binds with promoter region of has‐miR‐210‐3p in the presence of androgen.ConclusionsOur observations indicated that miRNAs 203a/210‐3p/5001‐5p regulate the androgen/AR/YAP‐induced PCa metastasis.
Bridging the Gap in the Structure–Function Paradigm of Enzymatic PET Degradation–Aromatic Residue Driven Balanced Interactions with Catalytic and Anchoring Subsite
Authors: James Anjima , Bhasi Anjitha , De Susmita 
Published: August 2024

AbstractUnderstanding all parameters contributing to enzyme activity is crucial in enzyme catalysis. For enzymatic PET degradation, this involves examining the formation of the enzyme‐PET complex. In IsPETase (WT), a PET‐degrading enzyme from Ideonella sakaiensis, mutating two non‐catalytic residues (DM) significantly enhances activity. Such mutations, depending on their position in the tertiary structure, fine‐tune enzyme function. However, detailed molecular insights into these mutations’ structure‐function relationship for PET degradation are lacking. This study characterizes IsPETase's catalytic ability compared to WT TfCut2 using molecular dynamics simulations and quantum mechanical methods. We explore the conformational landscape of the enzyme‐PET complex and quantify residue‐wise interaction energy. Notably, aromatic and hydrophobic residues Tyr, Trp, and Ile in the catalytic subsite S1, and aromatic Phe and polar Asn in the anchoring subsite S3, crucially optimize PET binding. These residues enhance PET specificity over non‐aromatic plastics. Our findings suggest that the balance between binding at subsite S1 and subsite S3, which is influenced by cooperative mutations, underlies catalytic activity. This balance shows a positive correlation with experimentally obtained kcat/Km values: WT TfCut2<WT IsPETase≪DM IsPETase.