bluuebunny/crossref_metadata_2025_split huggingface dataset:
An improved functional assay in blood spot to diagnose Barth syndrome using the monolysocardiolipin/cardiolipin ratio
Authors: Vaz Frédéric M. , van Lenthe Henk , Vervaart Martin A. T. , Stet Femke S. , Klinkspoor Johanne H. , Vernon Hilary J. , Goorden Susan M. I. , Houtkooper Riekelt H. , Kulik Willem , Wanders Ronald J. A. 
Published: August 2021

AbstractBarth syndrome is an X‐linked disorder characterized by cardiomyopathy, skeletal myopathy, and neutropenia, caused by deleterious variants in TAFAZZIN. This gene encodes a phospholipid‐lysophospholipid transacylase that is required for the remodeling of the mitochondrial phospholipid cardiolipin (CL). Biochemically, individuals with Barth syndrome have a deficiency of mature CL and accumulation of the remodeling intermediate monolysocardiolipin (MLCL). Diagnosis typically relies on mass spectrometric measurement of CL and MLCL in cells or tissues, and we previously described a method in blood spot that uses a specific MLCL/CL ratio as diagnostic biomarker. Here, we describe the evolution of our blood spot assay that is based on the implementation of reversed phase‐UHPLC separation followed by full scan high resolution mass spectrometry. In addition to the MLCL/CL ratio, our improved method also generates a complete CL spectrum allowing the interrogation of the CL fatty acid composition, which considerably enhances the diagnostic reliability. This addition negates the need for a confirmatory test in lymphocytes thereby providing a shorter turn‐around‐time while achieving a more certain test result. As one of the few laboratories that offer this assay, we also evaluated the diagnostic yield and performance from 2006 to 2021 encompassing the use of both the original and improved assay. In this period, we performed 796 diagnostic analyses of which 117 (15%) were characteristic of Barth syndrome. In total, we diagnosed 93 unique individuals with Barth syndrome, including three females, which together amounts to about 40% of all reported individuals with Barth syndrome in the world.
Diffusion Tensor Imaging Reveals Altered Topological Efficiency of Structural Networks in Type‐2 Diabetes Patients With and Without Mild Cognitive Impairment
Authors: Xiong Ying , Tian Tian , Fan Yang , Yang Shaolin , Xiong Xiaoxiao , Zhang Qiang , Zhu Wenzhen 
Published: August 2021

BackgroundSome patients with type 2 diabetes mellitus (T2DM) progress towards mild cognitive impairment (MCI), while some patients can always maintain normal cognitive function. Network topologic alterations at global and nodal levels between T2DM individuals with and without cognitive impairment may underlie the difference.PurposeTo investigate the topological alterations of the whole‐brain white matter (WM) structural connectome in T2DM patients with and without MCI and characterize its relationship with disease severity.Study TypeCross‐sectional and prospective study.SubjectsForty‐four (63.6% females) T2DM patients, 22 with mild cognitive impairment (DM‐MCI) and 22 with normal cognition (DM‐NC), and 34 (58.8% females) healthy controls (HC).Field Strength/Sequence3 T/diffusion tensor imaging.AssessmentGraph theoretical analysis was used to investigate the topological organization of the structural networks. The global topological properties and nodal efficiency were investigated and compared. Relationship between network metrics and clinical measurements was characterized.Statistical TestsStudent's t‐test, chi‐square test, ANOVA, partial correlation analyses, and multiple comparisons correction.ResultsThe global topological organization of WM networks was significantly disrupted in T2DM patients with cognitive impairment (reduced global and local efficiency and increased shortest path length) but not in those with normal cognition, compared with controls. The DM‐MCI group had significantly decreased network efficiency compared with the DM‐NC group. Compared with controls, decreased nodal efficiency was detected in three regions in DM‐NC group. More regions with decreased nodal efficiency were found in the DM‐MCI group. Altered global network properties and nodal efficiency of some regions were correlated with diabetic duration, HbA1c levels, and cognitive assessment scores.Data ConclusionThe more disrupted WM connections and weaker organized network are found in DM‐MCI patients relative to DM‐NC patients and controls. Network analyses provide information for the neuropathology of cognitive decline in T2DM patients. Altered nodal efficiency may act as potential markers for early detection of T2DM‐related MCI.Level of Evidence1Technical EfficacyStage 2
Self‐compassion in organizations: A review and future research agenda
Authors: Dodson Samantha J. , Heng Yu Tse 
Published: August 2021

SummaryBurgeoning organizational research has begun to explore how self‐compassion or the compassion individuals give to themselves in times of suffering may positively contribute to organizational outcomes. This review describes self‐compassion and its theoretical underpinnings in a workplace context, systematically reviews the current empirical literature on self‐compassion using samples of working adults, critically analyzes the current state of the literature, and provides recommendations for robust future research. In the process, we offer a dynamic, process‐based conceptualization of self‐compassion that aligns past research with current directions in organizational compassion and several suggestions for using a processual approach to improve theoretical and empirical rigor. We also guide future scholarly work surrounding self‐compassion in organizations by highlighting fundamental research questions that could advance our theoretical understanding of self‐compassion in organizational contexts.
Analytical database of Martian minerals (ADaMM): Project synopsis and Raman data overview
Authors: Veneranda Marco , Sanz‐Arranz Aurelio , Manrique Jose Antonio , Saiz Jesus , Garcia‐Prieto Clara , Pascual‐Sánchez Elena , Medina Jesus , Konstantinidis Menelaos , Lalla Emmanuel , Moral Andoni , Nieto Luis Miguel , Rull Fernando , Lopez‐Reyes Guillermo 
Published: August 2021

AbstractThe Mars2020/Perseverance and ExoMars/Rosalind Franklin rovers are both slated to return the first Raman spectra ever collected from another planetary surface, Mars. In order to optimize the rovers scientific outcome, the scientific community needs to be provided with tailored tools for data treatment and interpretation. Responding to this need, the purpose of the Analytical Database of Martian Minerals (ADaMM) project is to build an extended multianalytical database of mineral phases that have been detected on Mars or are expected to be found at the landing sites where the two rovers will operate. Besides the use of conventional spectrometers, the main objective of the ADaMM database is to provide access to data collected by means of laboratory prototypes simulating the analytical performances of the spectroscopic systems onboard the Mars 2020 and ExoMars rovers. Planned to be released to the public in 2022, ADaMM will also provide access to data treatment and visualization tools developed in the framework of the mentioned space exploration missions. As such, the present work seeks to provide an overview of the ADaMM online platform, spectral tools, and mineral collection. In addition to that, the manuscript describes the Raman spectrometers used to analyze the mineral collection and presents a representative example of the analytical performance ensured by the Raman prototypes assembled to simulate the Raman Laser Spectrometer (RLS) and SuperCam systems.
Identification and characterization of basic copper sulfates as mineral green pigments in Andean colonial mural paintings: Use of temperature‐controlled stage for the study of thermal induced antlerite degradation
Authors: Tomasini Eugenia P. , Costantini Ilaria , Rúa Landa Carlos , Guzmán Fernando , Pereira Magdalena , Castro Kepa , Siracusano Gabriela , Madariaga Juan Manuel , Maier Marta S. 
Published: August 2021

AbstractThis work describes the characterization of green pigments in wall paintings from the Andean churches of San José de Soracachi and Santiago de Callapa in Bolivia, located on an ancient colonial commercial route known as the Silver Route. To approach our goal, microsamples extracted from the mural paintings and mineral samples, from a mine of the same area, were studied by using micro‐Raman spectroscopy complemented with micro‐energy‐dispersive X‐ray fluorescence spectroscopy (μ‐EDXRF) and scanning electron microscopy with energy‐dispersive X‐ray spectroscopy (SEM‐EDS) analysis. The use of gypsum as the preparation layer was detected, and it suggestsa seccopainting technique. Antlerite, a basic copper sulfate, was identified suggesting a preference for its use as green pigment in the mural paintings of Andean churches that may be related to the local availability of this mineral. Indeed, this study revealed the presence of heterogeneous particles of the basic copper sulfate together with aluminosilicates, quartz and iron oxides among others, which points to a mineral origin of the green pigment because the same compounds were found in the samples taken in the mine. Because a phase transition has been noticed during the Raman analyses, due to a high power of the laser radiation, some tests have been carried out, varying the laser power, and coupling the Raman spectrometer to a temperature‐controlled stage to verify under which conditions the degradation of the mineral sample of antlerite occurs allowing in this way a correct characterization of basic copper sulfate pigments. These results contribute to the study of the Andean colonial artistic cultural heritage, and this information will be made available for the construction of a database of local pigments of mineral origin used in Andean colonial art.
Method validation and residue analysis of methoxyfenozide and metaflumizone in Chinese broccoli under field conditions by liquid chromatography with tandem mass spectrometry
Authors: Bi Yingying , Yao Wei , Han Lijun , Qiao Chengkui , Song Shuangyu , Qin Fayi , Dong Qin , Hao Xianghong , Xu Yanjun 
Published: August 2021

AbstractMethoxyfenozide and metaflumizone are insecticides used on Chinese broccoli to prevent insects and increase yield. However, the residues are potentially harmful to the environment and consumers. In this study, the quick, easy, cheap, effective, rugged, safe method with high‐performance liquid chromatography with tandem mass spectrometry was modified and validated for determination of methoxyfenozide and metaflumizone in Chinese broccoli. The clean‐up efficiency of different sorbents including C18, primary secondary amine, graphitized carbon black, and carbon nanofiber was compared. Recoveries of the validated method were 71.8–94.6% with relative standard deviations of 1.5–3.2% and the limits of quantification were 0.01 and 0.005 mg/kg for methoxyfenozide and metaflumizone, respectively. A storage stability test showed almost no degradation of methoxyfenozide in Chinese broccoli, however, the degradation rate of metaflumizone was 22.9% after 10‐wk storage at –20°C. In field trials in four producing regions, the dissipation of both methoxyfenozide and metaflumizone in Chinese broccoli was fast, with half‐lives of only 1.0–5.1 and 0.7–2.5 days, respectively. Terminal residues after application of the two pesticides were all below 1.0 mg/kg after 5 days.
Echocardiographic Characteristics and Contrast‐Enhanced Imaging of Intravenous Leiomyomatosis With Intracardiac Extension
Authors: Ma Hui , Niu Yicui , Yang Zihao , Zheng Minjuan 
Published: August 2021

ObjectivesIntravenous leiomyomatosis (IVL) is a histologically benign but biologically aggressive tumor. This study aimed to summarize the echocardiography and contrast‐enhanced ultrasound (CEU) characteristics of IVL to provide a basis for clinical diagnosis and therapy.MethodsFourteen IVL patients with uterus leiomyoma history (female, 46.4 ± 5.6 years) were enrolled in this study from March 2008 to December 2020 in our hospital. Preoperative imaging examination data were collected, including echocardiography computed tomography data; six patients also underwent CEU. All patients underwent successful resection, confirmed by histopathology.ResultsEchocardiographic characteristics: The mean sizes of intracardiac parts of IVL tumors were 54.0 ± 17.9 mm (length) and 24.6 ± 9.8 mm (width). IVL tumors exhibited two echocardiography types: isoechoic solid mass (71.4%, 10/14) and anechoic cystic conduits (28.6%, 4/14), with enlargements of the right atrium (57.1%,8/14), right ventricle (1 patient, 7.1%), and inferior vena cava (57.1%, 8/14). About 21.4% of the patients (3/14) had right ventricular dysfunction. Right heart obstruction was observed in 42.8% (6/14) of the patients. CEU characteristics: the solid mass type exhibited an earlier perfusion and lower perfusion intensity than the conduits type. CEU was helpful in determining origins and pathways: from the internal iliac vein (pathway I, 71.4%), from the ovarian vein (pathway II, 14.3%), or both (14.3%). The echocardiographic appearances of the 14 cases were consistent with the features of the resection specimens.ConclusionCombined echocardiography and CEU can provide a more valuable information for the diagnosis of IVL and essential basis for treatment.
Hyperspectral optical absorption closure experiment in complex coastal waters
Authors: Kostakis Ina , Twardowski Michael , Roesler Collin , Röttgers Rüdiger , Stramski Dariusz , McKee David , Tonizzo Alberto , Drapeau Susan 
Published: August 2021

AbstractAccurate measurements of absorption data are required for the development and validation of inversion algorithms for upcoming hyperspectral ocean color imaging sensors, such as the NASA Phytoplankton, Aerosol, Cloud, and ocean Ecosystem mission. This study aims to provide uncertainty estimates associated with leading approaches to measure hyperspectral absorption coefficients in complex coastal waters. Absorption spectra were collected at 12 different stations, all located in the Indian River Lagoon, Florida, USA, between 09 January 2017 and 13 January 2017. Measurements included spectral absorption coefficients in the visible range (400–700 nm) associated with dissolved, aCDOM, total particulate, ap, and total nonwater, anw, fractions, and were made both in situ and from discrete samples. Discrete sample approaches included dual‐beam spectrophotometer, liquid waveguide capillary cell, point‐source integrating cavity absorption meter (PSICAM) for dissolved matter absorption samples, and quantitative filter technique ICAM measurements and the dual‐beam spectrophotometer with center‐mounted integrating sphere filter pad technique, while the Turner Designs ICAM, and WET Labs AC‐s, and AC‐9 instruments were used to determine absorption coefficients in situ. The Gershun approach, determining absorption from measurement of the irradiance quartet with respect to depth was also assessed in situ. Measurement uncertainties and relative accuracies were quantified for each of these approaches. Results showed generally strong agreements between different discrete sample methods, with average percent absolute error %δabs < 7% for aCDOM and < 9% for ap. In situ approaches showed higher variability and reduced accuracy. For anw, %δabs deviation relative to PSICAM data was on average 12% to 20%. Results help identify remaining technological gaps and need for improvements in the different absorption measurement approaches.
A rare PALB2 germline variant causing G2/M cell cycle arrest is associated with isolated myelosarcoma in infancy
Authors: Beer Angelina , Beck Ricardo , Schedel Anne , von Bonin Malte , Meinel Jörn , Friedrich Ulrike Anne , Menzel Maria , Suttorp Meinolf , Brenner Sebastian , Fitze Guido , Lange Björn , Knöfler Ralf , Hauer Julia , Auer Franziska 
Published: August 2021

AbstractBackgroundIsolated myelosarcoma of infancy is a rare presentation of acute myelogenous leukaemia (AML). Because of its rarity and early onset in infancy underlying genetic predisposition is potentially relevant in disease initiation.Methods and ResultsWe report an oncologic emergency in an infant with thoracic and intraspinal aleukaemic myeloid sarcoma causing acute myelon compression and lower leg palsy. Whole‐exome sequencing of the patient's germline DNA identified a rare PALB2 (OMIM 610355) variant (p.A1079S), which is located in a domain critical for the gene's proper function within the homology‐directed repair pathway. In line with potential DNA damage repair defects mediated by the PALB2 deregulation, the patient's fibroblasts showed increased sensitivity towards radiation and DNA intercalating agents.ConclusionTherefore, we suggest PALB2 p.A1079S as a pathogenic variant potentially contributing to the here observed patient phenotype.
Replacement of Nitrite in Meat Products by Natural Bioactive Compounds Results in Reduced Exposure to N‐Nitroso Compounds: The PHYTOME Project
Authors: van Breda Simone G. , Mathijs Karen , Pieters Harm‐Jan , Sági‐Kiss Virág , Kuhnle Gunter G. , Georgiadis Panagiotis , Saccani Giovanna , Parolari Giovanni , Virgili Roberta , Sinha Rashmi , Hemke Gert , Hung Yung , Verbeke Wim , Masclee Ad A. , Vleugels‐Simon Carla B. , van Bodegraven Adriaan A. , de Kok Theo M. ,   the PHYTOME consortium
Published: August 2021

ScopeIt has been proposed that endogenously form N‐nitroso compounds (NOCs) are partly responsible for the link between red meat consumption and colorectal cancer (CRC) risk. As nitrite has been indicated as critical factor in the formation of NOCs, the impact of replacing the additive sodium nitrite (E250) by botanical extracts in the PHYTOME project is evaluated.Method and ResultsA human dietary intervention study is conducted in which healthy subjects consume 300 g of meat for 2 weeks, in subsequent order: conventional processed red meat, white meat, and processed red meat with standard or reduced levels of nitrite and added phytochemicals. Consumption of red meat products enriched with phytochemicals leads to a significant reduction in the faecal excretion of NOCs, as compared to traditionally processed red meat products. Gene expression changes identify cell proliferation as main affects molecular mechanism. High nitrate levels in drinking water in combination with processed red meat intake further stimulates NOC formation, an effect that could be mitigated by replacement of E250 by natural plant extracts.ConclusionThese findings suggest that addition of natural extracts to conventionally processed red meat products may help to reduce CRC risk, which is mechanistically support by gene expression analyses.
Microsatellite Variation in Yukon River Coho Salmon: Population Structure and Application to Mixed-Stock Analysis
Authors: Flannery Blair G. , Loges Randal G. , Wenburg John K. 
Published: August 2021

Abstract
Knowledge of population structure facilitates the effective management of species that are harvested in mixed-stock fisheries. In this study, we analyzed genetic variation at 19 microsatellite loci for 14 populations of Coho Salmon Oncorhynchus kisutch in the Yukon River. We then used this data to estimate the stock composition of adults that were harvested in a lower river test fishery. The Coho Salmon populations in the Yukon River exhibited a high degree of geographically based genetic structure (GST = 0.078), with a strong genetic disjunction between the lower and upper river populations. Further substructure was observed among the upper river populations, which allowed for the estimation of stock composition to areas within this region. Analyses involving simulated and real mixtures indicated that this level of divergence can be used to apportion Coho Salmon to regions and areas accurately (95–100%). The stock composition estimates for the test fishery revealed that the spawning migration was evenly divided (50:50) between the lower and upper river populations. The upper river populations generally had earlier migration timing, comprising 66% of the mixture in the beginning and 33% at the end. The largest component of the upper river region was Tanana at 44%, followed by Nenana at 5%, and Porcupine at 1%. While escapement monitoring is essential for the management of Coho Salmon, it is limited by funding shortfalls. However, mixed-stock analysis in conjunction with sonar enumeration can provide information on stock-specific proportions, abundances, and migration timings that can increase our knowledge and ability to manage Coho Salmon populations in the Yukon River.
Surface microenvironment optimization‐ induced robust oxygen reduction for neutral zinc‐air batteries
Authors: Liu Si , Cheng Han , Xia Jun , Wang Chun , Gui Renjie , Zhou Tianpei , Liu Hongfei , Peng Jing , Zhang Nan , Wang Wenjie , Chu Wangsheng , Wu HengAn , Wu Changzheng , Xie Yi 
Published: August 2021

AbstractNeutral zinc‐air batteries (ZABs) are promising candidates for the next‐generation power devices with considerably elongated lifetime comparing to conventional alkaline ZABs. However, neutral cathodic oxygen reduction reaction (ORR) is seriously limited by the mass transfer efficiency of hydroxyl due to insufficient interfacial chemical potential‐gradient between catalytic layer and electrolyte. Herein, we highlight that electrochemical oxidation‐induced surface microenvironment optimization could realize optimal chemical potential‐gradient around catalytic sites and bring outstanding neutral ORR activity. The electrodeposited sub‐nano Pt decorated surface‐microenvironment‐optimized Co2N samples (denoted as Pt‐SMO‐Co2N NWs) possessed 92 and 338 mV higher half‐wave potential than commercial Pt/C and pristine Co2N in 0.2 M PBS. As for neutral ZABs, Pt‐SMO‐Co2N NWs cathode delivers a power density of 67.9 mW*cm−2 and displays negligible decay after nearly 80 h stability test at 20 mA*cm−2. In‐depth characterization proposes that remarkable performance improvement originates from optimized microenvironment, which increases the surface chemical potential gradient and facilitates proton coupled electron transfer during ORR. We anticipated that such synergetic optimization of microenvironment and intrinsic activity of active sites is an effective strategy which may be extended to the catalytic reactions beyond ORR.Key points
We demonstrated surface microenvironment optimization via in‐situ electrochemical oxidation as an effective way to design highly active ORR catalysts.
Surface microenvironment optimization realized significantly enhanced ORR and Zn‐air battery performance in neutral media.
Based on ideal material platform, the key role of activating H2O and facilitating proton transfer process in ORR catalysis was revealed.
Leading and lagging indicators in fundraising management: A Canadian case study
Authors: Dougherty Christopher N. 
Published: August 2021

AbstractFundraisers, managers, and boards in the charitable sector are faced with an ongoing concern: how do they produce sustainable, predictable financial returns for their causes while minimizing the cost of fundraising? One way to address this is to improve the measurement of fundraising activities and this study asks how fundraising results should be communicated within organizations to support sustainability. This case study focuses on the fundraising program from one Canadian charity with a large, diversified fundraising program to examine how fundraisers can move beyond simple end‐of‐year financial ratios and implement one managerial technique, leading and lagging indicators, to improve long‐term financial performance. A literature review, internal interviews, and internal document review are used to identify 81 potential leading and lagging indicators that fundraisers can use to develop a suite of indicators that fit their context, activities, and goals and to identify potential challenges with implementing indicators. The role of organizational context and characteristics in selecting an appropriate suite of indicators is also discussed.