bluuebunny/crossref_metadata_2025_split huggingface dataset:
Class II Human Leukocyte Antigen Epitope Mismatch Predicts De Novo Donor‐Specific Antibody Formation After Liver Transplantation
Authors: Kubal Chandrashekhar A. , Mangus Richard , Ekser Burcin , Mihaylov Plamen , Ceballos Brian , Higgins Nancy , Chalasani Naga , Ghabril Marwan , Nephew Lauren , Lobashevsky Andrew 
Published: August 2018

Abstract
Formation of de novo donor‐specific antibodies (dn‐DSAs) has been associated with longterm immunologic complications after liver transplantation (LT). We hypothesized that human leukocyte antigen (HLA) epitope/eplet mismatch (MM) is a marker of immunogenicity and a risk factor for dn‐DSA formation. Sera from 80 LT recipients were prospectively screened for dn‐DSA by a Luminex single‐antigen test (One Lambda, Inc., Canoga Park, CA) at 1, 2, 3, 6, 12, 18, 24, and 36 months after LT. HLA typing of the recipients and donors was performed using polymerase chain reaction (PCR)–SSP and PCR‐SSOP Luminex low‐resolution methods (One Lambda, Inc.). The HLAMatchmaker computer algorithm was used for identification of MM eplets at HLA‐DRB1 and ‐DQA1/B1 loci. Luminex single‐antigen bead solid phase assay was used for antibody analysis. Standard immunosuppression included thymoglobulin‐rituximab induction and tacrolimus maintenance. There were 27 (34%) patients who developed dn‐DSA. There were no episodes of antibody‐mediated rejection, and 9 (11%) developed acute cellular rejection (ACR). A positive crossmatch status and a higher number of HLA‐A, ‐B, ‐DR, and ‐ABDR MMs were not associated with dn‐DSA formation. Patients developing dn‐DSA had a significantly higher number of total (38 ± 2.7 versus 28 ± 2.3; P = 0.01) and antibody‐verified (AbVer; 14 ± 1.1 versus 10 ± 1; P = 0.015) class II MM eplets. By a multivariate regression analysis, the number of class II MM eplets was strongly associated with risk of class II dn‐DSA formation (odds ratio [OR], 1.2; P < 0.01). Patients with ACR had a significantly higher number of total (20.2 ± 1.3 versus 13.9 ± 0.9; P < 0.01) as well as AbVer (10.7 ± 1.1 versus 7.5 ± 0.6; P = 0.03) class I MM eplets. In conclusion, donor‐recipient HLA epitope MM is associated with a risk of dn‐DSA formation and rejection after LT. However, further studies are required to evaluate the clinical utility of epitope matching in LT.
Average distances between points in graph‐directed self‐similar fractals
Authors: Olsen L. , Richardson A. 
Published: August 2018

AbstractWe study several distinct notions of average distances between points belonging to graph‐directed self‐similar subsets of . In particular, we compute the average distance with respect to graph‐directed self‐similar measures, and with respect to the normalised Hausdorff measure. As an application of our main results, we compute the average distance between two points belonging to the Drobot–Turner set with respect to the normalised Hausdorff measure, i.e. we compute
urn:x-wiley:0025584X:media:mana201600354:mana201600354-math-0003where s denotes the Hausdorff dimension of and is the s‐dimensional Hausdorff measure; here the Drobot–Turner set (introduced by Drobot & Turner in 1989) is defined as follows, namely, for positive integers N and m and a positive real number c, the Drobot–Turner set is the set of those real numbers for which any m consecutive base N digits in the N‐ary expansion of x sum up to at least c. For example, if , and , then our results show that
urn:x-wiley:0025584X:media:mana201600354:mana201600354-math-0011where is the unique positive real number such that .
Lignin‐g‐poly(acrylamide)‐g‐poly(diallyldimethyl‐ ammonium chloride): Synthesis, Characterization and Applications
Authors: Price Jacquelyn Tara , Gao Weijue , Fatehi Pedram 
Published: August 2018

AbstractThe search for a renewable substitute to petroleum‐based products has fueled increasing research on lignin, an under‐utilized product from pulping processes. In this work, lignin was copolymerized with acrylamide (AM) and diallyldimethylammonium chloride (DADMAC) under acidic conditions with Na2S2O8 as an initiator, generating a cationic water‐soluble lignin‐g‐P(AM)‐g‐P(DADMAC) copolymer. The optimal reaction conditions, using a 5×4 factorial design experiment, were determined to be an AM/DADMAC/lignin molar ratio of 5.5:2.4:1, 90 °C, 0.26 mol L−1 of lignin, and pH 2. Under the optimal reaction conditions, the resulting lignin‐g‐P(AM)‐g‐P(DADMAC) copolymer was 83 % soluble in an aqueous solution (at 10 g L−1) and at neutral pH. The copolymer had a charge density of 1.27 meq g−1, molecular weight of (1.33±0.08) ×106, an AM grafting ratio of 112 wt %, and a DADMAC grafting ratio of 20 wt %. In addition, the activation energy for producing this copolymer as well as the thermal and rheological properties of the copolymer were determined. The flocculation performance of lignin‐g‐P(AM)‐g‐P(DADMAC) copolymer was evaluated in a kaolin suspension, which showed that the lignin copolymer had a comparable flocculation efficiency with the synthetic analogue of P(AM)‐g‐P(DADMAC) at pH 6.
Thalassemia major phenotype caused by HB Zürich‐Albisrieden [α2 59(E8) Gly > Arg (HBA2:C.178G > C)] in a Brazilian child
Authors: Pedroso Gisele A. , Kimura Elza M. , Santos Magnun N.N. , Albuquerque Dulcinéia M. , Malimpensa Danaê , Jorge Susan E. , Verissimo Monica P.A. , Costa Fernando F. , Sonati Maria F. 
Published: August 2018

AbstractHemoglobin (Hb) Zürich‐Albisrieden (ZA) [α2 59(E8) Gly > Arg; HBA2:c.178G > C] is a rare and highly unstable α‐chain variant. A few simple and compound heterozygotes (αZAα/αα and –/αZAα, respectively) have been described so far in Switzerland and China. We describe here a case of homozygosity for the Hb ZA mutation (αZAα/αZAα) in a Brazilian child with severe congenital hemolytic anemia and ineffective erythropoiesis.
Two‐year‐old female with EBV‐positive diffuse large B‐cell lymphoma and subsequent CNS involvement with neurolymphomatosis
Authors: Bjornard Kari L. , Leventaki Vasiliki , Nichols Kim E. , Sandlund John T. , Prockop Susan , Ehrhardt Matthew J. 
Published: August 2018

AbstractWe describe a case of Epstein‐Barr virus (EBV) positive, diffuse large B‐cell lymphoma in a 2‐year‐old female who went on to develop relapsed/refractory central nervous system (CNS) disease, manifesting as cranial nerve neurolymphomatosis. Although her atypical presentation was thought to be associated with an immune deficiency, extensive work‐up was negative. Despite subsequent treatment with third‐party EBV‐specific cytotoxic T‐lymphocytes, she died of progressive disease. This case report raises questions as to whether tailored treatment approaches should be considered for atypical presentations of pediatric lymphoma (e.g., CNS and virus‐associated).
Synthesis, Characterization and Physical Properties of Tantalum Pentoxide Reinforced Ni‐Fe Alloy Matrix Nanocomposites
Authors: Chaudhari Alok Kumar , Singh Vijai Bahadur 
Published: August 2018

AbstractSoft magnetic Ni−Fe/Ta2O5 nanocomposites were synthesized by electro‐codeposition on a copper substrate from an ethylene glycol bath. Formation of the composites, tantalum oxide particle content and structure evolution were investigated using X‐ray diffractometer (XRD), transmission electron microscope (TEM), scanning electron microscope (SEM) and energy dispersive X‐ray analyzer (EDAX). Maximum particle content of the deposits was up to 5.7 wt% and clusters of Ta2O5 particles were found on the coating surface. This appears beneficial for improving the corrosion resistance of the coatings. With increasing current density, relative texture coefficient of the coatings shifted from (111) to (200) and followed (220) crystallographic plane. Although crystallite size was recorded approximately 15 nm in the entire current density range studied, remarkable enhancement in the microhardness of the Ni−Fe/Ta2O5 nanocomposite coatings was noticed (up to 870 HV) owing to reinforcement of ceramic particles.
Vanadium Nitride as an Efficient and Stable Promotor for Pd‐Catalyzed Methanol Electrooxidation in Alkaline Media
Authors: Meng Kai , Zheng Zhilin , Jia Zhijun , Ma Dan , Liu Lujing , Wang Yi , Qi Tao 
Published: August 2018

AbstractPd/C electrocatalysts containing VN with different morphology (particles (Pd‐VN(particles)/C) and plates (Pd‐VN(plates)/C)) were prepared by a simple reduction with sodium borohydride and their electrocatalytic performance for methanol oxidation was investigated. The Pd‐VN(particles)/C and Pd‐VN(plates)/C performed better activity, stronger resistance to CO poisoning and higher stability for methanol oxidation compared with Pd/C by the measurements of cyclic voltammograms (CVs), CO stripping voltammetry and chronoamperometry (CA), respectively. The X‐ray photoelectron spectroscopy (XPS), linear sweep voltammetry (LSV) and electrochemical impedance spectrums (EIS) tests were conducted for exploring the role of VN. It is found that the promotion effects of VN can be explained by a bifunctional mechanism and the increase in metallic Pd in electrocatalysts containing VN. Furthermore, the different promotive extents between the VN with different morphology are attributed to the differences of the crystallinity and contact situation of Pd and VN.
Mesoporous Co–CoO@NC Micro‐Disk Derived from ZIF‐9 as Bifunctional Catalyst for Lithium‐Oxygen Batteries
Authors: Chen Xiang , Chen Chunguang , Zhang Xiuhui , Huang Tao , Yu Aishui 
Published: August 2018

AbstractMetal and metal oxides are considered as effective catalysts owing to their excellent oxygen reduction and oxygen evolution reaction activity. However, their low electrical conductivity, the collapse of the structure during the cycles and agglomeration in thermal treatment, which can degrade the electrochemical properties, are still puzzles for researchers. Herein, we present a facile method to synthesize mesoporous N‐doped carbon matrix modified with cobalt and cobalt oxide (Co–CoO@NC) micro‐disk derived from zeolitic imidazolate frameworks, alleviating the agglomeration of the cobalt nanoparticles to form CoO with abundant oxygen vacancies and introducing nitrogen in the carbon matrix with strong metal‐N‐doped carbon bridging bonds. The porous structure shows numerous mesoporous with diameters of 4–7 nm and an excellent specific surface area as high as 739.4 m2 g−1. The lithium‐oxygen batteries based on Co–CoO@NC electrode show improved electrochemical performance, with a high initial discharge capacity up to 5582 mAh g−1 at 0.05 mA cm−2 and preferable cycling stability with a cut‐off specific capacity of 500 mAh g−1 at 0.1 mA cm−2. The improved performance is mainly ascribed to the synergic effect of the porous structure, CoO and strong metal‐N‐doped carbon bridging bonds, leading to the excellent OER and ORR activities.
Hierarchical Assembly of Nanocellulose‐Based Filaments by Interfacial Complexation
Authors: Zhang Kaitao , Liimatainen Henrikki 
Published: August 2018

AbstractIn the present study, interfacial complexation spinning of oppositely charged cellulose‐materials is applied to fabricate hierarchical and continuous nanocellulose based filaments under aqueous conditions by using cationic cellulose nanocrystals with different anionic celluloses including soluble sodium carboxymethyl cellulose and insoluble 2,2,6,6‐tetramethylpiperidinyl‐1‐oxy radical‐oxidized cellulose nanofibers and dicarboxylated cellulose nanocrystals (DC‐CNC). The morphologies of the wet and dry nanocellulose based filaments are further investigated by optical and electron microscopy. All fabricated continuous nanocellulose based filaments display a hierarchical structure similar to the natural cellulose fibers in plant cells. As far as it is known, this is not only the first report about the fabrication of nanocellulose based filaments by interfacial complexation of cationic CNCs with anionic celluloses but also the first demonstration of fabricating continuous fibers directly from oppositely charged nanoparticles by interfacial nanoparticle complexation (INC). This INC approach may provide a new route to design continuous filaments from many other oppositely charged nanoparticles with tailored characteristics.
A NASICON‐Type Positive Electrode for Na Batteries with High Energy Density: Na4MnV(PO4)3
Authors: Chen Fan , Kovrugin Vadim M. , David Rénald , Mentré Olivier , Fauth François , Chotard Jean‐Noël , Masquelier Christian 
Published: August 2018

AbstractThe mixed Mn2+/V3+ Na‐super‐ionic‐conductor (NASICON) cathode material Na4MnV(PO4)3 is prepared by solid‐state reaction at 800 °C under argon. When used as a positive electrode in Na batteries, this material can exchange three electrons for two transition metals, that is, yielding a high gravimetric capacity of 156 mAh g−1 on charge when the upper cutoff voltage is set to 4.3 V versus Na+/Na. Operando X‐ray diffraction during battery operation is performed and it is shown that the mechanisms of Na+ insertion/extraction are single or biphasic depending on the electrochemical cycling conditions.
Adventures in Defining Roles of Oxygenases in the Regulation of Protein Biosynthesis
Authors: Walport Louise J. , Schofield Christopher J. 
Published: August 2018

AbstractThe 2‐oxoglutarate (2OG) dependent oxygenases were first identified as having roles in the post‐translational modification of procollagen in animals. Subsequently in plants and microbes, they were shown to have roles in the biosynthesis of many secondary metabolites, including signalling molecules and the penicillin/cephalosporin antibiotics. Crystallographic studies of microbial 2OG oxygenases and related enzymes, coupled to DNA sequence analyses, led to the prediction that 2OG oxygenases are widely distributed in aerobic biology. This personal account begins with examples of the roles of 2OG oxygenases in antibiotic biosynthesis, and then describes efforts to assign functions to other predicted 2OG oxygenases. In humans, 2OG oxygenases have been found to have roles in small molecule metabolism, as well as in the epigenetic regulation of protein and nucleic acid biosynthesis and function. The roles and functions of human 2OG oxygenases are compared, focussing on discussion of their substrate and product selectivities. The account aims to emphasize how scoping the substrate selectivity of, sometimes promiscuous, enzymes can provide insights into their functions and so enable therapeutic work.
Enhancement of NAD(H) pool for formation of oxidized biochemicals in Escherichia coli
Authors: Han Qi , Eiteman Mark A 
Published: August 2018

Abstract
The NAD+/NADH ratio and the total NAD(H) play important roles for whole-cell biochemical redox transformations. After the carbon source is exhausted, the degradation of NAD(H) could contribute to a decline in the rate of a desired conversion. In this study, methods to slow the native rate of NAD(H) degradation were examined using whole-cell Escherichia coli with two model oxidative NAD+-dependent biotransformations. A high phosphate concentration (50 mM) was observed to slow NAD(H) degradation. We also constructed E. coli strains with deletions in genes coding several enzymes involved in NAD+ degradation. In shake-flask experiments, the total NAD(H) concentration positively correlated with conversion of xylitol to l-xylulose by xylitol 4-dehydrogenase, and the greatest conversion (80%) was observed using MG1655 nadR nudC mazG/pZE12-xdh/pCS27-nox. Controlled 1-L batch processes comparing E. coli nadR nudC mazG with a wild-type background strain demonstrated a 30% increase in final l-xylulose concentration (5.6 vs. 7.9 g/L) and a 25% increase in conversion (0.53 vs. 0.66 g/g). MG1655 nadR nudC mazG was also examined for the conversion of galactitol to l-tagatose by galactitol 2-dehydrogenase. A batch process using 15 g/L glycerol and 10 g/L galactitol generated over 9.4 g/L l-tagatose, corresponding to 90% conversion and a yield of 0.95 g l-tagatose/g galactitol consumed. The results demonstrate the value of minimizing NAD(H) degradation as a means to improve NAD+-dependent biotransformations.
Molecular genetics of a cohort of 635 cases of phenylketonuria in a consanguineous population
Authors: Shirzadeh Tina , Saeidian Amir Hossein , Bagherian Hamideh , Salehpour Shadab , Setoodeh Aria , Alaei Mohammad Reza , Youssefian Leila , Samavat Ashraf , Touati Andrew , Fallah Mohammad‐Sadegh , Vahidnezhad Hassan , Karimipoor Morteza , Azadmehr Sarah , Raeisi Marzieh , Bandehi Sarhadi Ameneh , Zafarghandi Motlagh Fatemeh , Jamali Mojdeh , Zeinali Zahra , Abiri Maryam , Zeinali Sirous ,   Additional individual contributors
Published: August 2018

AbstractPhenylketonuria (PKU) is an inborn error of amino acid metabolism caused by mutations in the phenylalanine hydroxylase (PAH) gene, characterized by intellectual deficit and neuropsychiatric complications in untreated patients with estimated frequency of about one in 10,000 to 15,000 live births. PAH deficiency can be detected by neonatal screening in nearly all cases with hyperphenylalaninemia on a heel prick blood spot. Molecular testing of the PAH gene can then be performed in affected family members. Herein, we report molecular study of 635 patients genetically diagnosed with PKU from all ethnicities in Iran. The disease‐causing mutations were found in 611 (96.22%) of cases. To the best of our knowledge, this is the most comprehensive molecular genetics study of PKU in Iran, identifying 100 distinct mutations in the PAH gene, including 15 previously unreported mutations. Interestingly, we found unique cases of PKU with uniparental disomy, germline mosaicism, and coinheritance with another Mendelian single‐gene disorder that provides new insights for improving the genetic counseling, prenatal diagnosis (PND), and/or pre‐implantation genetic diagnosis (PGD) for the inborn error of metabolism group of disorders.
Late‐onset Pompe disease in France: molecular features and epidemiology from a nationwide study
Authors: Semplicini Claudio , Letard Pascaline , De Antonio Marie , Taouagh Nadjib , Perniconi Barbara , Bouhour Françoise , Echaniz‐Laguna Andoni , Orlikowski David , Sacconi Sabrina , Salort‐Campana Emmanuelle , Solé Guilhem , Zagnoli Fabien , Hamroun Dalil , Froissart Roseline , Caillaud Catherine , Laforêt Pascal ,   French Pompe Study Group
Published: August 2018

AbstractPompe disease (PD) is caused by a deficiency of lysosomal acid α‐glucosidase resulting from mutations in theGAAgene. The clinical spectrum ranges from a rapidly fatal multisystemic disorder (classic PD, onset < 1 year) to a milder adult onset myopathy. The aims of this study were to characterize theGAAmutations, to establish the disease epidemiology, and to identify potential genotype‐phenotype correlations in French late‐onset PD patients (onset ≥ 2 years) diagnosed since the 1970s. Data were collected from the two main laboratories involved in PD diagnosis and from the French Pompe registry. Two hundred forty‐six patients (130 females and 116 males) were included, with a mean age at diagnosis of 43 years. Eighty‐three different mutations were identified in theGAAgene, among which 28 were novel. These variants were spread all over the sequence and included 42 missense (one affecting start codon), 8 nonsense, 15 frameshift, 14 splice mutations, 3 small in‐frame deletions, and one large deletion. The common c.‐32‐13T>G mutation was detected in 151/170 index cases. Other frequent mutations included the exon 18 deletion, the c.525del, and the missense mutations c.1927G>A (p.Gly643Arg) and c.655G>A (p.Gly219Arg). Patients carrying the c.‐32‐13T>G mutation had an older mean age at onset than patients non‐exhibiting this mutation (36 versus 25 years). Patients with the same genotype had a highly variable age at onset. We estimated the frequency of late‐onset PD in France around 1/69,927 newborns. In conclusion, we characterized the French cohort of late‐onset PD patients through a nationwide study covering more than 40 years.
Fibroblast growth factor 21 as a biomarker for long‐term complications in organic acidemias
Authors: Molema F. , Jacobs E. H. , Onkenhout W. , Schoonderwoerd G. C. , Langendonk J. G. , Williams Monique 
Published: August 2018

AbstractBackgroundThere is increasing evidence that long‐term complications in organic acidemias are caused by impaired mitochondrial metabolism. Currently, there is no specific biomarker to monitor mitochondrial dysfunction in organic acidemias. Serum fibroblast growth factor 21 (FGF‐21) is a biomarker for mitochondrial disease and could be a candidate to monitor mitochondrial function in the deleterious course of disease.MethodsData of 17 patients with classical organic acidemias (11 propionic acidemia (PA), four methylmalonic acidemia (MMA) and two isovaleric acidemia (IVA) patients) were included. The clinical course was evaluated; metabolic decompensations and long‐term complications were correlated with plasma FGF‐21 levels. Cardiomyopathy, prolonged QT interval, renal failure, and optic neuropathy were defined as long‐term complications.ResultsPatients ages ranged from 16 months up to 32 years. Serious long‐term complications occurred in eight patients (five PA and three MMA patients). In MMA and PA patients plasma FGF‐21 levels during stable metabolic periods were significantly higher in patients with long‐term complications (Mdn = 2556.0 pg/ml) compared to patients without (Mdn = 287.0 pg/ml). A median plasma FGF‐21 level above 1500 pg/ml during a stable metabolic period, measured before the occurrence of long‐term complications, had a positive predictive value of 0.83 and a negative predictive value of 1.00 on long‐term complications in MMA and PA patients.ConclusionThis study demonstrates the potential role of FGF‐21 as a biomarker for long‐term complications in classical organic acidemias, attributed to mitochondrial dysfunction.
Bayesian latent time joint mixed‐effects model of progression in the Alzheimer's Disease Neuroimaging Initiative
Authors: Li Dan , Iddi Samuel , Thompson Wesley K. , Rafii Michael S. , Aisen Paul S. , Donohue Michael C. ,   Alzheimer's Disease Neuroimaging Initiative
Published: August 2018

AbstractIntroductionWe characterize long‐term disease dynamics from cognitively healthy to dementia using data from the Alzheimer's Disease Neuroimaging Initiative.MethodsWe apply a latent time joint mixed‐effects model to 16 cognitive, functional, biomarker, and imaging outcomes in Alzheimer's Disease Neuroimaging Initiative. Markov chain Monte Carlo methods are used for estimation and inference.ResultsWe find good concordance between latent time and diagnosis. Change in amyloid positron emission tomography shows a moderate correlation with change in cerebrospinal fluid tau (ρ = 0.310) and phosphorylated tau (ρ= 0.294) and weaker correlation with amyloid‐β 42 (ρ = 0.176). In comparison to amyloid positron emission tomography, change in volumetric magnetic resonance imaging summaries is more strongly correlated with cognitive measures (e.g.,ρ= 0.731 for ventricles and Alzheimer's Disease Assessment Scale). The average disease trends are consistent with the amyloid cascade hypothesis.DiscussionThe latent time joint mixed‐effects model can (1) uncover long‐term disease trends; (2) estimate the sequence of pathological abnormalities; and (3) provide subject‐specific prognostic estimates of the time until onset of symptoms.
Imperfect Alternatives: Institutional Choice and the Reform of Investment Law
Authors: Puig Sergio , Shaffer Gregory 
Published: August 2018

AbstractThis Article applies the theory of comparative institutional analysis to evaluate the trade-offs associated with alternative mechanisms for resolving investment disputes. We assess the trade-offs in light of the principle of accountability under the rule of law, which underpins the goals of fairness, efficiency, and peace that are attributed to investment law. The Article makes two recommendations: first, reforms should address complementarity between domestic and international institutions; second, institutional choices should respond to the different contexts that states face.