bluuebunny/crossref_metadata_2025_split huggingface dataset:
A Dual‐Functional Photosensitizer for Ultraefficient Photodynamic Therapy and Synchronous Anticancer Efficacy Monitoring
Authors: Gao Yuting , Wang Xiuxia , He Xuewen , He Zhenyan , Yang Xiang , Tian Sidan , Meng Fanling , Ding Dan , Luo Liang , Tang Ben Zhong 
Published: June 2019

AbstractA dual‐functional photosensitizer that demonstrates exceptional photodynamic therapy (PDT) efficacy while simultaneously self‐monitoring the therapeutic response in real time is reported here. Possessing an ultrahigh 1O2 quantum yield of 98.6% in water, the photosensitizer TPCI can efficiently induce cell death in a series of carcinoma cells (IC50 values less than 300 × 10−9 m) upon irradiation with an extremely low fluence (460 nm, 4 mW cm−2 for 10 min). In addition, TPCI can self‐monitor cell death in real time. It is weakly fluorescent in living cells before irradiation and lights up the nuclei concomitantly with cell death during PDT treatment by binding with chromatin to activate its aggregation‐induced emission, attributed to its strong binding affinity with DNA. In vivo studies using mouse models bearing H22 and B16F10 tumor cells validate the ultraefficient PDT efficacy of TPCI as well as the precise real‐time noninvasive readout of the tumor response from the beginning of cancer treatment. The dual‐functional TPCI serves as an excellent candidate for single‐agent photodynamic theranostics, and this work represents a new paradigm for the development of molecules with multiple intrinsic functions for future self‐reporting medical applications.
Spontaneous Phase Separation of Poly(3‐hexylthiophene)s with Different Regioregularity for a Stretchable Semiconducting Film
Authors: Gao Chun Yan , Pei Mingyuan , Choi Hyoung Jin , Yang Hoichang 
Published: June 2019

AbstractHighly regioregular (RR) poly(3‐hexylthiophene)s PHTs are known to exhibit excellent electrical properties in comparison to chemically identical but regiorandom (rr) PHTs. In this study, distinct RR (97% and 55%)‐graded PHTs are subjected to solution blending to spontaneously separate the high‐RR PHT chains from the low‐RR PHT media and develop highly conjugated nanodomains in both solution and film. In the spun‐cast blend films, the rr PHT matrix imparts sufficient deformability of the channel layer required for stretchable organic thin‐film transistors (OTFTs), compared to neat RR PHTs and blends with a deformable polymer. OTFTs including RR PHT/rr PHT blend films show excellent hole mobility (µ) values up to 0.13 cm2 V−1 s−1, surpassing that of the best RR PHT films (0.026 cm2 V−1 s−1) fabricated by ultrasound solution pretreatment. Furthermore, a 50% stretched RR PHT/rr PHT film maintains ≈55% of its µ value at no strain, while RR PHT films show a sudden decrease in µ even at 10% stretch. The simple blending approach imparts deformability to π‐conjugated polymer films for application in stretchable OTFTs.
Clinical features and mutational analysis in 114 young children with Wilson disease from South China
Authors: Li Xiuzhen , Lu Zhikun , Lin Yunting , Lu Xinshuo , Xu Yi , Cheng Jing , Shao Yongxian , Su Xueying , Liu Zongcai , Sheng Huiying , Cai Yanna , Li Taolin , Zhou Zhizi , Tan Jingwen , Liu Hongsheng , Huang Yonglan , Liu Li , Zeng Chunhua 
Published: June 2019

AbstractWilson disease (WD) is a rare autosomal recessive disorder caused by mutations in the ATP7B gene. Clinical features and mutational analysis of Chinese children with WD at early age were rarely described. Herein, we retrospectively examined 114 children with WD at the mean of 5.9 years old age at diagnosis. Eight patients developed acute liver failure at mean age of 9.7 years old, 4 of whom died. Among the 114 patients, 86.0% were presymptomatic with isolated elevation of transaminases at diagnosis, 99.1% had decreased ceruloplasmin, and 68.4% had urinary copper excretion over 100 μg/24 hr. Bi‐allele pathogenic ATP7B mutations were identified in all patients. Among the 60 mutations detected, 10 were novel, including 7 missense mutations (p.I566N, p.T704I, p.C980F, p.G1030 V, p.A1096Q, p.L1327P, and p.L1373F), 1 nonsense mutation (p.K866X), 1 small insertion (p.Y44LfsX2), and 1 small deletion (p.R1118PfsX10). The most frequent mutations were p.R778L, p.P992L, and p.I1148T, which affected 27.2, 25.4, and 20.2% of the 114 WD children, respectively. The patients carrying p.R778L presented a higher rate of acute liver failure than the patients without p.R778L (9.7% vs. 4.8%). These results will be helpful in establishing early diagnosis of WD at the gene level, offering beneficial information for genetic counseling and providing clues to genotype/phenotype correlation of ATP7B mutations.
Sensitive detection of FGFR1 N546K mosaic mutation in patient with encephalocraniocutaneous lipomatosis and pilocytic astrocytoma
Authors: Kordacka Joanna , Zakrzewski Krzysztof , Gruszka Renata , Witusik‐Perkowska Monika , Taha Joanna , Sikorska Beata , Liberski Paweł P. , Zakrzewska Magdalena 
Published: June 2019

AbstractEncephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous disorder, with only about 100 cases reported worldwide. It is characterized by congenital lesions of the eye, skin, and central nervous system. Only recently, potential causative FGFR1 point mutations have been identified in brain tumors and cultured skin biopsies from patients with this condition. Here, we analyzed the molecular status of a patient with ECCL and a coexisting pilocytic astrocytoma with detected FGFR1 N546K mutation. The presence of the alteration in both affected and unaffected tissues has been evaluated using Sanger sequencing and droplet digital polymerase chain reaction (ddPCR) technique. The ddPCR analysis showed differential distribution of the alteration in all specimens, including unaffected and untreated samples. Therefore, we confirm that FGFR1 N546K is a plausible causative mutation of ECCL patients and could be associated with a risk of brain tumor development. We also show the usefulness of sensitive ddPCR method for detection of low levels of autosomal mosaic mutation in blood or swabs. We suggest that utilization of this method may improve the diagnostic process, especially when targeted therapies are considered.
Severe Noonan syndrome phenotype associated with a germline Q71R MRAS variant: a recurrent substitution in RAS homologs in various cancers
Authors: Suzuki Hisato , Takenouchi Toshiki , Uehara Tomoko , Takasago Satoshi , Ihara Satoshi , Yoshihashi Hiroshi , Kosaki Kenjiro 
Published: June 2019

AbstractActivation of the RAS pathway through either the activation of genes that accelerate the pathway or the suppression of genes that inhibit the pathway leads to a group of disorders collectively referred to as RASopathies. The key molecules of the RAS pathway are KRAS, HRAS, and NRAS. Mutations in these three RAS homolog genes have been shown to be associated with RASopathies. Recently, two patients with a Noonan syndrome phenotype were shown to carry mutations in the yet another RASopathy gene, MRAS (muscle RAS oncogene homolog). Here, we report a patient with a severe Noonan syndrome phenotype associated with a germline Q71R MRAS variant, which represents a recurrent substitution in RAS homologs in various cancers. The patient's dysmorphic features included relative macrocephaly, a down‐slanted palpebral fissure, hypertelorism, a depressed nasal bridge, and low‐set ears with thick lobes; these facial features are strongly associated with RASopathy. We confirmed that the MRAS gene represents a causative gene for RASopathy.
An Enigmatic Small Neosuchian Crocodyliform from the Woodbine Formation of Texas
Authors: Noto Christopher R. , Drumheller Stephanie K. , Adams Thomas L. , Turner Alan H. 
Published: June 2019

ABSTRACTNew discoveries at the Arlington Archosaur Site (AAS), a Cenomanian (Late Cretaceous) locality in north‐central Texas, are filling gaps in our knowledge of mid‐Cretaceous Appalachian ecosystems, which remain poorly characterized. The AAS is notable because it preserves a diverse crocodyliform record. As seen in other sites that preserve four or more crocodyliform taxa, the species present at the AAS exhibit different snout shapes and body sizes, indicating that this high diversity of sympatric species was likely sustainable due to niche partitioning. Here we describe Scolomastax sahlsteini gen. et sp. nov., a new species of crocodyliform from the AAS, currently known from a partial right mandibular ramus. This species differs from other crocodyliforms in possessing features associated with durophagy or omnivory, including a shortened mandible, reduced tooth count, heterodonty, a dorsally expanded surangular, and enlarged attachments for jaw adductor muscles. Our phylogenetic analysis places this new taxon within Eusuchia as a member of Paralligatoridae and sister taxon to Paralligator gradilifrons. Scolomastax sahlsteini extends the record of paralligatorids into the Late Cretaceous of North America. This discovery represents the first appearance of this clade on the poorly known landmass of Appalachia, supporting a biogeographic connection between North America and Asia in the Early Cretaceous prior to completion of the Western Interior Seaway. However, relationships among other endemic crocodyliforms and tree instability within Paralligatoridae suggest further analysis is needed to resolve phylogenetic and biogeographic relationships (http://zoobank.org/urn:lsid:zoobank.org:pub:DC114471‐6687‐4BB5‐8FAE‐96F7278B1DAF). Anat Rec, 303:801–812, 2020. © 2019 Wiley Periodicals, Inc.
Fernando de Castro: Cajal's Man on the Peripheral Nervous System
Authors: Ros‐Bernal Francisco , de Castro Fernando 
Published: June 2019

ABSTRACTSantiago Ramón y Cajal developed his initial scientific career working alone. After the publication of his opus magna (“Textura del sistema nervioso del hombre y los vertebrados”) and the general recognition of the scientific environments that crystallized with the concession of the International Moscow Prize (1900), the Spanish Government decided to officially support Cajal with a laboratory and the first salaries to pay collaborators. Is then when the Spanish Neurological School births: in 1902, Francisco Tello is the first one to be incorporated. With new additions, Cajal's work is complimented in new aspects, including Neuropathologies. Fernando de Castro is one of his youngest direct disciples, one of the closest and more beloved. Fernando de Castro worked from 1916 in Cajal's lab, until the death of El Maestro. He was specially committed by Cajal to unravel different aspects of the structure of the peripheral ganglia: sensitive and vegetative. Afterward, Fernando de Castro described by first time the nature of arterial chemoreceptors in the carotid body. While trying to confirm his anatomical description with physiological demonstrations, and accumulating delays because of scientific decision and the sociopolitical circumstances in Spain, Corneille Heymans was awarded with the Nobel Prize in Physiology or Medicine 1938 for his contributions to the knowledge of cardiorespiratory reflexes. The Karolinska Institutet forgot Heinrich Hering and Fernando de Castro in their decision. Undoubtedly, Fernando de Castro was the most important disciple of Cajal working in the different structures of the peripheral nervous system, and this work is now reviewed here. Anat Rec, 303:1206–1214, 2020. © 2019 American Association for Anatomy
Layer‐by‐Layer Assembly: Recent Progress from Layered Assemblies to Layered Nanoarchitectonics
Authors: Ariga Katsuhiko , Ahn Eungjin , Park Minju , Kim Byeong‐Su 
Published: June 2019

AbstractAs an emerging concept for the development of new materials with nanoscale features, nanoarchitectonics has received significant recent attention. Among the various approaches that have been developed in this area, the fixed‐direction construction of functional materials, such as layered fabrication, offers a helpful starting point to demonstrate the huge potential of nanoarchitectonics. In particular, the combination of nanoarchitectonics with layer‐by‐layer (LbL) assembly and a large degree of freedom in component availability and technical applicability would offer significant benefits to the fabrication of functional materials. In this Minireview, recent progress in LbL assembly is briefly summarized. After introducing the basics of LbL assembly, recent advances in LbL research are discussed, categorized according to physical, chemical, and biological innovations, along with the fabrication of hierarchical structures. Examples of LbL assemblies with graphene oxide are also described to demonstrate the broad applicability of LbL assembly, even with a fixed material.
β‐Selective Glycosylation by Using O‐Aryl‐Protected Glycosyl Donors
Authors: Otsuka Yuji , Yamamoto Toshihiro , Fukase Koichi 
Published: June 2019

AbstractNew glycosyl donors have been developed that contained several para‐substituted O‐aryl protecting groups and their stereoselectivity for the glycosylation reaction was evaluated. A highly β‐selective glycosylation reaction was achieved by using thioglycosides that were protected by 4‐nitrophenyl (NP) groups, which were introduced by using the corresponding diaryliodonium triflate. Analysis of the stereoselectivities of several glycosyl donors indicated that the β‐glycosides were obtained through an SN2‐type displacement from the corresponding α‐glycosyl triflate. The NP group could be removed by reduction of the nitro group and acylation, followed by oxidation with ceric ammonium nitrate (CAN).
Outcome of patients aged 60‐75 years with newly diagnosed secondary acute myeloid leukemia: A single‐institution experience
Authors: Bertoli Sarah , Tavitian Suzanne , Bories Pierre , Luquet Isabelle , Delabesse Eric , Comont Thibault , Sarry Audrey , Huguet Françoise , Bérard Emilie , Récher Christian 
Published: June 2019

AbstractA recent phase 3 trial showed that outcome of older patients with secondary acute myeloid leukemia (AML) may be improved by a liposomal encapsulation of cytarabine and daunorubicin (CPX‐351). This phase 3 study represents a unique example of prospective data in this rare subgroup providing basis for comparison with real life data. Here, we retrospectively assessed characteristics and outcome of patients aged 60‐75 years with secondary or therapy‐related AML in real life. Out of 218 patients that fulfilled CPX‐351 study criteria, 181 patients (83.0%) received antileukemic treatment either intensive chemotherapy (n = 121) or hypomethylating agents (HMA, n = 60). As compared with patients treated by chemotherapy, HMA‐treated patients were older, had lower WBC, more often AML with antecedent myelodysplastic syndrome and adverse cytogenetic risk. In chemotherapy‐treated patients, the complete response rate was 69%, median overall survival (OS) was 11 months whereas 3‐year and 5‐year OS was 21% and 17%, respectively. In HMA‐treated patients, the complete response rate was 15%, median OS was 11 months whereas 3‐year and 5‐year OS was 15% and 2%, respectively. In conclusion, although outcome of older patients with high‐risk AML is very poor, a significant proportion of patients treated by standard intensive chemotherapy but not HMA are long‐term survivors.
Anal adenocarcinoma: Treatment outcomes and trends in a rare disease entity
Authors: Wegner Rodney E. , White Richard J. , Hasan Shaakir , Raj Moses , Monga Dulabh , Finley Gene , Kirichenko Alexander V. , McCormick James 
Published: June 2019

AbstractImportancePrimary Adenocarcinoma of the anus is a rare disease with a poor prognosis and thus tends to have a more aggressive treatment algorithm, typically involving a surgical approach. Prior to 2001, a few retrospective studies outlined improved outcomes with the incorporation of surgery with chemoradiation. However, since the publication of these studies, advancement in radiotherapy modalities and imaging have left the question of improved outcomes while reserving surgery for salvage.ObjectiveWe conducted this National Cancer Database (NCDB)‐driven retrospective study to analyze treatment trends and outcomes in the current time from 2004 to 2015 with respect to chemoradiation and surgery.DesignRetrospective NCDB tumor registry data review—using propensity score‐adjusted multivariable analyses for survival.SettingDatabase review.ParticipantsWe selected for patients listed in the NCDB with AJCC stage 1‐3 anal adenocarcinoma diagnosed between 2004 and 2015 and selected out patients with undocumented/stage 4 disease, those with radiation outside the pelvis, not treated with systemic therapy and patients lost to follow‐up.Exposure(s)None.Main outcomes and measuresOverall survival and use of surgery in the up‐front management of anal adenocarcinoma.ResultsOf the 1729 patients eligible in this study, 1028 were treated with surgery as up‐front management and 701 had definitive chemoradiation. Median overall survival for all patients was 55 months with a 5‐year survival rate of 55%. Patients treated without surgery had worse overall survival, median survival of 45 months compared to 87 months (P < 0.0001) with 5‐year survival rates of 42% and 55% in favor of incorporation of surgery. Analysis across patients treated with surgery alone, surgery followed by adjuvant chemoradiation, neoadjuvant chemoradiation followed by surgery, and chemoradiation alone had median survival rates of 78, 83, 92, and 46 months, respectively. Propensity score‐adjusted multivariable analysis identified older age, grade 3, high comorbidity score, and lack of surgery as predictive of worse outcome.Conclusions and RelevanceThe results of the NCDB analysis indicate improved overall survival with the incorporation of surgery into the initial management of anal adenocarcinoma when compared to chemoradiation alone, despite the omission of surgery in up to 50% of the cases logged. Our results corroborate earlier studies published prior to the year 2000 for surgery to be included in the definitive management of anal adenocarcinoma.
Second primary malignancies in postpolycythemia vera and postessential thrombocythemia myelofibrosis: A study on 2233 patients
Authors: Mora Barbara , Rumi Elisa , Guglielmelli Paola , Barraco Daniela , Maffioli Margherita , Rambaldi Alessandro , Caramella Marianna , Komrokji Rami , Gotlib Jason , Kiladjian Jean Jacques , Cervantes Francisco , Devos Timothy , Palandri Francesca , De Stefano Valerio , Ruggeri Marco , Silver Richard T. , Benevolo Giulia , Albano Francesco , Cavalloni Chiara , Pietra Daniela , Barbui Tiziano , Rotunno Giada , Cazzola Mario , Vannucchi Alessandro Maria , Giorgino Toni , Passamonti Francesco 
Published: June 2019

AbstractPatients with myeloproliferative neoplasms (MPN) are known to have higher incidence of nonhematological second primary malignancies (SPM) compared to general population. In the MYSEC study on 781 secondary myelofibrosis (SMF) patients, the incidence of SPM after SMF diagnosis resulted 0.98/100 patient‐years. When including non‐melanoma skin cancers (NMSC), the incidence arose to 1.56/100 patient‐years. In SMF, JAK inhibitor treatment was associated only with NMSC occurrence. Then, we merged the MYSEC cohort with a large dataset of PV and ET not evolving into SMF. In this subanalysis, we did not find any correlation between SPM and SMF occurrence. These findings highlight the need of studies aimed at identifying MPN patients at higher risk of SPM.
GDF15 induced apoptosis and cytotoxicity in A549 cells depends on TGFBR2 expression
Authors: Tarfiei Ghorban Ali , Shadboorestan Amir , Montazeri Hamed , Rahmanian Narges , Tavosi Gholamreza , Ghahremani Mohammad Hossein 
Published: June 2019

GDF15 plays a paradoxical role during carcinogenesis; it inhibits tumour growth in the early stages and promotes tumour cell proliferation in the late stages of cancer. Besides, GDF15 can induce apoptosis in some cancer cells including A549 but not in some others. Moreover, as a potential receptor for GDF15, TGFBR2 is inactivated during carcinogenesis in many types of cancers, and it is not present in cells with no GDF15 induced apoptosis. Thus, we tested whether GDF15 overexpression and/or TGFBR2 silencing can affect the GDF15 induced apoptosis in A549 cells. The full and mature forms of GDF15 were cloned and overexpressed in A549 cells. The TGFBR2 was silenced using specific siRNA and confirmed by real‐time PCR. Results indicated that overexpression of full and mature forms of GDF15 as well as TGFBR2 knocked down reduced A549 cell viability in 24 and 48 hours. Flow cytometric analysis of annexin V/PI indicated induction of apoptosis in A549 cells by overexpression of GDF15 or silencing TGFBR2. Interestingly, the silencing of TGFBR2 inhibited the GDF15 induced cytotoxicity and apoptosis in A549 cells. Overexpression of GDF15 activated caspase‐9 and caspase‐3 and inhibited ERK1/2 and p38 phosphorylation in A549 cells. TGFBR2 knocked down inhibited GDF15 effects on caspases, ERK1/2, and p38MAPK activation. Our results indicated that the effect of GDF15 on apoptosis and activation of MAPK in A549 cells depends on TGFBR2 expression. These findings may point to mechanisms in which GDF15 exerts dual effect during carcinogenesis with regard to TGFBR2 expression.Significance of the studyGDF15 plays a tumour suppressor or promotor roles during carcinogenesis. The expression of GDF15 induced cytotoxicity, apoptosis, and inhibition of MAPK in A549 cells. All these effects were blocked by silencing TGFBR2 expression. These findings may point to mechanisms in which GDF15 exerts dual effect during carcinogenesis with regard to TGFBR2 expression.
Isobutene from Ethanol: Describing the Synergy between In2O3 and m‐ZrO2
Authors: Zonetti Priscila C. , Bridi Vivian L. , Gonzalez Guilherme G. , Moreira Carla R. , Alves Odivaldo C. , de Avillez Roberto R. , Appel Lucia G. 
Published: June 2019

AbstractThe performances of In2O3/ZrO2, ZrO2 and In2O3 were evaluated in the synthesis of isobutene from ethanol in one‐step. These catalysts were characterized by XRD, EPR, TPD‐H2O, TPD‐NH3, TPD‐ethanol and pyridine adsorption. The In2O3/ZrO2 catalyst is a promising catalytic system for the synthesis of isobutene from ethanol. The catalytic tests showed that acetone, hydrogen and carbon dioxide are the main co‐products of this synthesis. The In2O3/ZrO2 catalyst can promote the synthesis of isobutene due to the inter‐diffusion process between In2O3 and ZrO2. This phenomenon generates, among others, the Zr insertion and a high concentration of anionic vacancies in the In2O3 lattice. This increases the In2O3/ZrO2 redox properties leading to a high activity of this catalyst for the acetone generation, a key intermediate of the isobutene synthesis. These changes also promote the acid and basic properties of In2O3/ZrO2, which are in charge of the acetaldehyde synthesis, acetone condensation and, finally, dehydration of the intermediates of the isobutene synthesis.