bluuebunny/crossref_metadata_2025 huggingface dataset:
Asymmetric C‐Alkylation by the S‐Adenosylmethionine‐Dependent Methyltransferase SgvM
Authors: Sommer‐Kamann Christina , Fries Alexander , Mordhorst Silja , Andexer Jennifer N. , Müller Michael
Published: March 2017

S‐Adenosylmethionine‐dependent methyltransferases (MTs) play a decisive role in the biosynthesis of natural products and in epigenetic processes. MTs catalyze the methylation of heteroatoms and even of carbon atoms, which, in many cases, is a challenging reaction in conventional synthesis. However, C‐MTs are often highly substrate‐specific. Herein, we show that SgvM from Streptomyces griseoviridis features an extended substrate scope with respect to the nucleophile as well as the electrophile. Aside from its physiological substrate 4‐methyl‐2‐oxovalerate, SgvM catalyzes the (di)methylation of pyruvate, 2‐oxobutyrate, 2‐oxovalerate, and phenylpyruvate at the β‐carbon atom. Chiral‐phase HPLC analysis revealed that the methylation of 2‐oxovalerate occurs with R selectivity while the ethylation of 2‐oxobutyrate with S‐adenosylethionine results in the S enantiomer of 3‐methyl‐2‐oxovalerate. Thus SgvM could be a valuable tool for asymmetric biocatalytic C‐alkylation reactions.
A Facile Steam Reforming Strategy to Delaminate Layered Carbon Nitride Semiconductors for Photoredox Catalysis
Authors: Yang Pengju , Ou Honghui , Fang Yuanxing , Wang Xinchen
Published: March 2017

The delamination of layered crystals that produces single or few‐layered nanosheets while enabling exotic physical and chemical properties, particularly for semiconductor functions in optoelectronic applications, remains a challenge. Here, we report a facile and green approach to prepare few‐layered polymeric carbon nitride (PCN) semiconductors by a one‐step carbon/nitrogen steam reforming reaction. Bulky PCN, obtained from typical precursors including urea, melamine, dicyandiamide, and thiourea, are exfoliated into few‐layered nanosheets, while engineering its surface carbon vacancies. The unique sheet structures with strengthened surface properties endow PCNs with more active sites, and an increased charge separation efficiency with a prolonged charge lifetime, drastically promoting their photoredox performance. After an assay of a H2 evolution reaction, an apparent quantum yield of 11.3 % at 405 nm was recorded for the PCN nanosheets, which is much higher than those of PCN nanosheets. This delamination method is expandable to other carbon‐based 2D materials for advanced applications.
Regioselective α‐Addition of Deconjugated Butenolides: Enantioselective Synthesis of Dihydrocoumarins
Authors: Wu Bo , Yu Zhaoyuan , Gao Xiang , Lan Yu , Zhou Yong‐Gui
Published: March 2017

The enantioselective α‐addition of deconjugated butenolides has rarely been exploited, in contrast to the well‐studied γ‐addition of deconjugated butenolides. In this study, an unprecedented asymmetric α‐addition/transesterification of deconjugated butenolides with ortho‐quinone methides generated in situ afforded a series of functionalized 3,4‐dihydrocoumarins containing two contiguous stereogenic centers with excellent diastereo‐ and enantioselectivity. DFT calculations suggested that the rarely observed regioselectivity was due to the distortion energy that resulted from the interaction between the nucleophilic dienolate and the electrophilic ortho‐quinone methide.
Computational Study of Oxidation of Guanine by Singlet Oxygen (1Δg) and Formation of Guanine:Lysine Cross‐Links
Authors: Thapa Bishnu , Munk Barbara H. , Burrows Cynthia J. , Schlegel H. Bernhard
Published: March 2017

Oxidation of guanine in the presence of lysine can lead to guanine–lysine cross‐links. The ratio of the C4, C5 and C8 crosslinks depends on the manner of oxidation. Type II photosensitizers such as Rose Bengal and methylene blue can generate singlet oxygen, which leads to a different ratio of products than oxidation by type I photosensitizers or by one electron oxidants. Modeling reactions of singlet oxygen can be quite challenging. Reactions have been explored using CASSCF, NEVPT2, DFT, CCSD(T), and BD(T) calculations with SMD implicit solvation. The spin contamination in open‐shell calculations were corrected by Yamaguchi's approximate spin projection method. The addition of singlet oxygen to guanine to form guanine endo‐ peroxide proceeds step‐wise via a zwitterionic peroxyl intermediate. The subsequent barrier for ring closure is smaller than the initial barrier for singlet oxygen addition. Ring opening of the endoperoxide by protonation at C4−O is followed by loss of a proton from C8 and dehydration to produce 8‐oxoGox. The addition of lysine (modelled by methylamine) or water across the C5=N7 double bond of 8‐oxoGox is followed by acyl migration to form the final spiro products. The barrier for methylamine addition is significantly lower than for water addition and should be the dominant reaction channel. These results are in good agreement with the experimental results for the formation of guanine–lysine cross‐links by oxidation by type II photosensitizers.
Field‐Induced Slow Magnetic Relaxation of GdIII Complex with a Pt−Gd Heterometallic Bond
Authors: Yoshida Takefumi , Cosquer Goulven , Izuogu David Chukwuma , Ohtsu Hiroyoshi , Kawano Masaki , Lan Yanhua , Wernsdorfer Wolfgang , Nojiri Hiroyuki , Breedlove Brian K. , Yamashita Masahiro
Published: March 2017

Heterometallic Gd−Pt complexes ([Gd2Pt3(H2O)2(SAc)12] (SAc=thioacetate), [Y1.4Gd0.6Pt3(H2O)2(SAc)12], and [Gd2Pt3(H2O)6(SAc)12]⋅7 H2O have been synthesized. The crystal structures and DFT calculations indicated a Gd−Pt heretometallic bond. Single‐crystal ESR spectra determined the direction of magnetic anisotropy as direction of the Gd−Pt bond. In other words, the Gd−Pt bond dictates the direction of magnetic anisotropy. The heterometallic Gd−Pt bond lowers the symmetry of the Gd ion, splitting the Kramers doublet in a dc field. Thus, we observed clear field‐induced slow magnetic relaxation of [Y1.4Gd0.6Pt3(H2O)2(SAc)12] up to 36 K. The relaxation process was determined to be a direct process.
Inorganic SnIP‐Type Double Helices in Main‐Group Chemistry
Authors: Baumgartner Maximilian , Weihrich Richard , Nilges Tom
Published: March 2017

Inspired by the synthesis of the first atomic‐scale double‐helix semiconductor SnIP, this study deals with the question of whether more atomistic, inorganic double‐helix compounds are accessible. With the aid of quantum chemical calculations, we have identified 31 candidates by a homoatomic substitution in MXPn, varying the Group 14 M‐element from Si to Pb, the Group 17 X‐element from F to I and replacing the pnictide (Pn) phosphorus by arsenic. The double‐helical structure of SnIP has been used as the starting model for all candidates and the electronic structure and vibrational spectra were determined within the framework of density functional theory (DFT). Varying the outer MX or the inner Pn helix led to the conclusion that iodide‐ and bromide‐containing MXPn compounds show similar structures to SnIP. Here, the calculations indicate interesting effects for electronic band‐gap tuning. For the highly polarized fluorides, a segregation of the helices to more complex MX substructures is predicted.
Quantifying the Effects of Halogen Bonding by Haloaromatic Donors on the Acceptor Pyrimidine
Authors: Ellington Thomas L. , Reves Peyton L. , Simms Briana L. , Wilson Jamey L. , Watkins Davita L. , Tschumper Gregory S. , Hammer Nathan I.
Published: March 2017

The effects of intermolecular interactions by a series of haloaromatic halogen bond donors on the normal modes and chemical shifts of the acceptor pyrimidine are investigated by Raman and NMR spectroscopies and electronic structure computations. Halogen‐bond interactions with pyrimidine's nitrogen atoms shift normal modes to higher energy and upfield shift 1H and 13C NMR peaks in adjacent nuclei. This perturbation of vibrational normal modes is reminiscent of the effects of hydrogen bonded networks of water, methanol, or silver on pyrimidine. The unexpected observation of vibrational red shifts and downfield 13C NMR shifts in some complexes suggests that other intermolecular forces such as π interactions are competing with halogen bonding. Natural bond orbital analyses indicate a wide range of charge transfer is possible from pyrimidine to different haloaromatic donors and computed halogen bond binding energies can be larger than a typical hydrogen bond. These results emphasize the importance in strategic selection of substituents and electron withdrawing groups in developing supramolecular structures based on halogen bonding.
Neuregulin1 fine‐tunes pre‐, post‐, and perisynaptic neuromuscular junction development
Authors: Wang Jiajing , Song Fei , Loeb Jeffrey A.
Published: March 2017

Background: Neuromuscular junction (NMJ) development is a multistep process mediated by coordinated interactions between the nerve terminal, target muscle, and perisynaptic Schwann cell that require constant back‐and‐forth communication. Retrograde and anterograde growth and differentiation factors have been postulated to participate in this communication. While neuregulin1 (NRG1) has been shown to be potent anterograde signal that activates acetylcholine receptor (AChR) transcription and clustering in vitro, its roles in NMJ development in vivo remain elusive. Results: Using the model of chicken embryo, we measured the effects of NRG1 signaling during NMJ development in ovo using quantitative, sequential measures of AChR cluster size and density, pre‐ and postsynaptic apposition, and the alignment of perisynaptic Schwann cells. Using in ovo electroporation at early stages and a targeted soluble neuregulin antagonist through all developmental stages, we found soluble NRG1 regulates AChR cluster density and size at the earliest stage prior to nerve‐AChR cluster contact. Once the nerve contacts with muscle AChRs, NRG1 has pronounced effects on presynaptic specialization and on the alignment of perisynaptic Schwann cells at endplates. Conclusion: These findings suggest that, while NRG1 may not be critical for overall development, it appears to be important in fine‐tuning pre‐, post‐, and perisynaptic development of the NMJ. Developmental Dynamics 246:368–380, 2017. © 2017 Wiley Periodicals, Inc.
Periodontitis and bone metabolism in patients with advanced heart failure and after heart transplantation
Authors: Schulze‐Späte Ulrike , Mizani Iman , Salaverry Kristina Rodriguez , Chang Jaime , Wu Christina , Jones Meaghan , Kennel Peter J. , Brunjes Danielle L. , Choo Tse‐Hwei , Kato Tomoko S. , Mancini Donna , Grbic John , Schulze P. Christian
Published: March 2017

Aims

Heart failure (HF) is a multi­organ, pro‐inflammatory syndrome that impairs bone metabolism. Pro‐inflammatory cytokines and bone catabolism enhance periodontal disease, a local inflammatory, bacteria‐induced disease that causes bone loss and periodontal soft tissue destruction.

Methods and results

Medical and dental examinations were performed on patients with HF (n = 39), following heart transplantation (post‐HTx, n = 38) and controls (n = 32). Blood, saliva, and gingival crevicular fluid were analysed for bone metabolism and inflammation markers. HF average New York Heart Association classification was III. Average time since HTx was 1414 days. Pro‐inflammatory tumour necrosis factor‐alpha was higher in HF and HTx as compared with controls (P < 0.05). Both HF and HTx participants had higher levels of bone resorption marker C‐terminal telopeptide and parathyroid hormone with subjects in the HF group having the highest serum levels of all groups (P ≤ 0.05). In contrast, 25‐hydroxyvitamin D was lowest in HF. HF patients had greater clinical attachment loss, cumulative pockets depth (greater than 3 mm) and probing depth (P < 0.05) as compared with controls. Cumulative pockets depth correlated significantly with measures of the inflammatory burden, β‐glucuronidase in saliva (r = 0.4863, P < 0.01), interleukin‐1b in saliva (r = 0.5149, P < 0.01), and gingival crevicular fluid (r = 0.6056, P < 0.001) in HF. However, adjustment of periodontal results for measures of oral hygiene (plaque, bleeding on probing), systemic 25‐hydroxyvitamin D, and race attenuated significant differences between groups.

Conclusions

Patients with HF exhibit more severe periodontal disease associated with increased bone turnover markers when compared with control patients. However, local and systemic factors may account for this association and should be evaluated in future studies.
Neoadjuvant chemotherapy in geriatric head and neck cancers
Authors: Patil Vijay M. , Noronha Vanita , Joshi Amit , Ramaswamy Anant , Dhumal Sachin , Juvekar Shashikant , Arya Supreeta , Mahajan Abhishek , Chaturvedi Pankaj , D'Cruz Anil , Bhattacharjee Atanu , Prabhash Kumar
Published: March 2017

Background

The purpose of this study was to present our findings on the treatment completion rates and outcomes in geriatric patients with head and neck cancer treated with neoadjuvant chemotherapy followed by definitive therapy.

Methods

Geriatric patients with locally advanced head and neck cancer who received neoadjuvant chemotherapy were selected for this analysis. Overall survival (OS) and progression‐free survival (PFS) were estimated.

Results

Forty‐six of 49 patients completed neoadjuvant chemotherapy (93.9%). The compliance to local treatment was 73.3% and the median OS was 49.9 months (95% confidence interval [CI] = 22.0–77.8 months) in patients who received neoadjuvant chemotherapy for organ preservation (n = 15). In patients receiving neoadjuvant chemotherapy for technically unresectable disease, the corresponding figures were 82.06% and 9.0 months (95% CI = 5.9–12.1 months), respectively.

Conclusion

Individualized neoadjuvant chemotherapy protocols in geriatric patients have high compliance rates to treatment and the median OS in geriatric patients treated for organ preservation is similar to that of young patients. © 2017 Wiley Periodicals, Inc. Head Neck 39: 886–892, 2017
Characteristics of cloud cluster over the steep southern slopes of the Himalayas observed by CloudSat
Authors: Chen Yilun , Fu Yunfei , Xian Tao , Pan Xiao
Published: March 2017

ABSRACT

A cloud cluster (CC) dataset is presented using CloudSat Cloud Profile Radar (CPR) observations from June to August, between 2006 and 2010. The CCs were defined by grouping contiguous cloud pixels using a defined criteria, including reflectivity, ‘cloud mask’, and topographic information. The characteristics of identified CCs were then analysed. Based on the complex topography of the southern Himalayas, four adjacent regions were selected, including the flat Gangetic Plains (FGP), the foothills of the Himalayas (FHH), the steep slope of the southern Himalayas (SSSH), and the Himalayan‐Tibetan Plateau (HTPT). The characteristics of the CCs in the four regions were different, and the distribution of the cloud‐top height gradually changed from bimodal (3 and 15 km) over the FGP and FHH, to unimodal (7–9 km) over the HTPT. From the plain to the Plateau, the average cloud‐top height and cloud‐base height increased after an initial decrease. The maximum reflectivity increased from −6 (plain) to 2 dBZ (slope areas), and then decreased to −2 dBZ (Plateau), which suggests that the CCs may produce precipitation on the slope. Maximum CCs occurred over the FHH. The characteristics of the CCs also showed semidiurnal variation, which may have been affected by the variation of the monsoon and the circulation over the mountain valley. According to the physical principles of the cloud‐type definitions from ISCCP, high‐level cirrus, deep convective CCs, and mid‐ to low‐level CCs were categorized. As the elevation increased, the cloud‐top height of the mid‐ to low‐level CCs also significantly increased to 8 km, whereas the cloud‐top height of the high‐level cirrus clouds decreased. Our results suggest that the different characteristics of CCs may be caused by a strong upward motion interacting with the terrain of the Himalayas.
Structural modeling of a novel SLC38A8 mutation that causes foveal hypoplasia
Authors: Toral Marcus A. , Velez Gabriel , Boudreault Katherine , Schaefer Kellie A. , Xu Yu , Saffra Norman , Bassuk Alexander G. , Tsang Stephen H. , Mahajan Vinit B.
Published: February 2017

Background

Foveal hypoplasia (FH) in the absence of albinism, aniridia, microphthalmia, or achromatopsia is exceedingly rare, and the molecular basis for the disorder remains unknown. FH is characterized by the absence of both the retinal foveal pit and avascular zone, but with preserved retinal architecture. SLC38A8 encodes a sodium‐coupled neutral amino acid transporter with a preference for glutamate as a substrate. SLC38A8 has been linked to FH. Here, we describe a novel mutation to SLC38A8 which causes FH, and report the novel use of OCT‐angiography to improve the precision of FH diagnosis. More so, we used computational modeling to explore possible functional effects of known SLC38A8 mutations.

Methods

Fundus autofluorescence, SD‐OCT, and OCT‐angiography were used to make the clinical diagnosis. Whole‐exome sequencing led to the identification of a novel disease‐causing variant in SLC38A8. Computational modeling approaches were used to visualize known SLC38A8 mutations, as well as to predict mutation effects on transporter structure and function.

Results

We identified a novel point mutation in SLC38A8 that causes FH. A conclusive diagnosis was made using OCT‐angiography, which more clearly revealed retinal vasculature penetrating into the foveal region. Structural modeling of the channel showed the mutation was near previously published mutations, clustered on an extracellular loop. Our modeling also predicted that the mutation destabilizes the protein by altering the electrostatic potential within the channel pore.

Conclusion

Our results demonstrate a novel use for OCT‐angiography in confirming FH, and also uncover genotype–phenotype correlations of FH‐linked SLC38A8 mutations.
Multi‐Stakeholder Initiatives for Sustainable Development Goals: The Importance of Interlocutors
Authors: Fowler Alan , Biekart Kees
Published: March 2017

This article argues that the complex multi‐stakeholder arrangements anticipated for implementing Sustainable Development Goals call for a distinct type of host: an interlocutor. This central idea arises from new comparative research on multi‐stakeholder initiatives (MSIs) undertaken in four countries: Costa Rica, Indonesia, Kenya and Kyrgyzstan. This work adds a detailed dimension to meta‐studies on conditions for success and practical guides for establishing and running MSIs. It begins to fill a significant gap in knowledge by analysing the attributes and competencies required for effectively orchestrating MSIs as well as illuminating their relative significance over time. The context is an anticipated expansion in demand for finely tuned and skilled hosting of Sustainable Development Goals‐inspired MSIs. This task will probably be more complicated than MSIs associated with climate change and Millennium Development Goals, both of which saw business on the side lines with uneven attention paid to the principle of local ownership. Recognising and investing in interlocution as a pivotal role can increase the performance of internationally inspired MSIs which, to date, have a mixed but generally poor record of effectiveness. © 2017 The Authors Public Administration and Development Published by John Wiley & Sons Ltd.
The influence of a radical structure on the kinetics of photopolymerization
Authors: Kabatc Janina
Published: March 2017

A radical initiation ability of new initiating systems in photopolymerization of 2‐ethyl‐2‐(hydroxymethyl)‐1,3‐propanediol triacrylate has been investigated and presented. The evaluation of alkyltriphenyl‐ and tetraalkylborates, iodonium salts, N‐alkoxypyridinium salts, maleimides, phthalimides, 1,3,5‐triazine derivatives and others as a free radical source in combination with suitable photosensitizer for radical polymerization of triacrylate is described. It is assumed that the photochemical decomposition of a coinitiator molecule results in formation of free radicals, which further initiate polymerization. The order of activity of free radical sources on kinetic of photopolymerization was also presented. Different initiator activity can be explained by the difference in the decomposition rate constant and the reactivity of radicals formed toward the double bond of monomer. © 2017 Wiley Periodicals, Inc. J. Polym. Sci., Part A: Polym. Chem. 2017, 55, 1575–1589
Rapid quantification and isotopic analysis of dissolved sulfur species
Authors: Smith Derek A. , Sessions Alex L. , Dawson Katherine S. , Dalleska Nathan , Orphan Victoria J.
Published: March 2017

Rationale

Dissolved sulfur species are of significant interest, both as important substrates for microbial activities and as key intermediaries in biogeochemical cycles. Species of intermediate oxidation state such as sulfite, thiosulfate, and thiols are of particular interest but are notoriously difficult to analyze, because of low concentrations and rapid oxidation during storage and analysis.

Methods

Dissolved sulfur species are reacted with monobromobimane which yields a fluorescent bimane derivative that is stable to oxidation. Separation by Ultra‐Performance Liquid Chromatography (UPLC) on a C18 column yields baseline resolution of analytes in under 5 min. Fluorescence detection (380 nm excitation, 480 nm emission) provides highly selective and sensitive quantitation, and Time‐of‐Flight Mass Spectrometry (TOF‐MS) is used to quantify isotopic abundance, providing the ability to detect stable isotope tracers (either 33S or 34S).

Results

Sulfite, thiosulfate, methanethiol, and bisulfide were quantified with on‐column detection limits of picomoles (μM concentrations). Other sulfur species with unshared electrons are also amenable to analysis. TOF‐MS detection of 34S enrichment was accurate and precise to within 0.6% (relative) when sample and standard had similar isotope ratios, and was able to detect enrichments as small as 0.01 atom%. Accuracy was validated by comparison to isotope‐ratio mass spectrometry. Four example applications are provided to demonstrate the utility of this method.

Conclusions

Derivatization of aqueous sulfur species with bromobimane is easily accomplished in the field, and protects analytes from oxidation during storage. UPLC separation with fluorescence detection provides low‐μM detection limits. Using high‐resolution TOF‐MS, accurate detection of as little as 0.01% 34S label incorporation into multiple species is feasible. This provides a useful new analytical window into microbial sulfur cycling. Copyright © 2017 John Wiley & Sons, Ltd.
Cover Image, Volume 9, Issue 1
Authors: Hadas Yoav , Katz Michael G. , Bridges Charles R. , Zangi Lior
Published: March 2017

The cover image, by Lior Zangi et al., is based on the Focus Article Modified mRNA as a therapeutic tool to induce cardiac regeneration in ischemic heart disease, DOI: 10.1002/wsbm.1367.

image
Clinico‐pathological Correlation of Thyroid Nodule Ultrasound and Cytology Using the TIRADS and Bethesda Classifications
Authors: Singaporewalla R. M. , Hwee J. , Lang T. U. , Desai V.
Published: March 2017

Background

Clinico‐pathological correlation of thyroid nodules is not routinely performed as until recently there was no objective classification system for reporting thyroid nodules on ultrasound. We compared the Thyroid Imaging Reporting and Data System (TIRADS) of classifying thyroid nodules on ultrasound with the findings on fine‐needle aspiration cytology (FNAC) reported using the Bethesda System.

Methods

A retrospective analysis of 100 consecutive cases over 1 year (Jan–Dec 2015) was performed comparing single‐surgeon‐performed bedside thyroid nodule ultrasound findings based on the TIRADS classification to the FNAC report based on the Bethesda Classification. TIRADS 1 (normal thyroid gland) and biopsy‐proven malignancy referred by other clinicians were excluded. Benign‐appearing nodules were reported as TIRADS 2 and 3. Indeterminate or suspected follicular lesions were reported as TIRADS 4, and malignant‐appearing nodules were classified as TIRADS 5 during surgeon‐performed bedside ultrasound. All the nodules were subjected to ultrasound‐guided FNAC, and TIRADS findings were compared to Bethesda FNAC Classification.

Results

Of the 100 cases, 74 were considered benign or probably benign, 20 were suspicious for malignancy, and 6 were indeterminate on ultrasound. Overall concordance rate with FNAC was 83% with sensitivity and specificity of 70.6 and 90.4%, respectively. The negative predictive value was 93.8%.

Conclusion

It is essential for clinicians performing bedside ultrasound thyroid and guided FNAC to document their sonographic impression of the nodule in an objective fashion using the TIRADS classification and correlate with the gold standard cytology to improve their learning curve and audit their results.
Expanding the phenotype in argininosuccinic aciduria: need for new therapies
Authors: Baruteau Julien , Jameson Elisabeth , Morris Andrew A. , Chakrapani Anupam , Santra Saikat , Vijay Suresh , Kocadag Huriye , Beesley Clare E. , Grunewald Stephanie , Murphy Elaine , Cleary Maureen , Mundy Helen , Abulhoul Lara , Broomfield Alexander , Lachmann Robin , Rahman Yusof , Robinson Peter H. , MacPherson Lesley , Foster Katharine , Chong W. Kling , Ridout Deborah A. , Bounford Kirsten McKay , Waddington Simon N. , Mills Philippa B. , Gissen Paul , Davison James E.
Published: March 2017

Objectives

This UK‐wide study defines the natural history of argininosuccinic aciduria and compares long‐term neurological outcomes in patients presenting clinically or treated prospectively from birth with ammonia‐lowering drugs.

Methods

Retrospective analysis of medical records prior to March 2013, then prospective analysis until December 2015. Blinded review of brain MRIs. ASL genotyping.

Results

Fifty‐six patients were defined as early‐onset (n = 23) if symptomatic < 28 days of age, late‐onset (n = 23) if symptomatic later, or selectively screened perinatally due to a familial proband (n = 10). The median follow‐up was 12.4 years (range 0–53). Long‐term outcomes in all groups showed a similar neurological phenotype including developmental delay (48/52), epilepsy (24/52), ataxia (9/52), myopathy‐like symptoms (6/52) and abnormal neuroimaging (12/21). Neuroimaging findings included parenchymal infarcts (4/21), focal white matter hyperintensity (4/21), cortical or cerebral atrophy (4/21), nodular heterotopia (2/21) and reduced creatine levels in white matter (4/4). 4/21 adult patients went to mainstream school without the need of additional educational support and 1/21 lives independently. Early‐onset patients had more severe involvement of visceral organs including liver, kidney and gut. All early‐onset and half of late‐onset patients presented with hyperammonaemia. Screened patients had normal ammonia at birth and received treatment preventing severe hyperammonaemia. ASL was sequenced (n = 19) and 20 mutations were found. Plasma argininosuccinate was higher in early‐onset compared to late‐onset patients.

Conclusions

Our study further defines the natural history of argininosuccinic aciduria and genotype–phenotype correlations. The neurological phenotype does not correlate with the severity of hyperammonaemia and plasma argininosuccinic acid levels. The disturbance in nitric oxide synthesis may be a contributor to the neurological disease. Clinical trials providing nitric oxide to the brain merit consideration.
Shakespeare and the Admiral's Men
Authors: Rutter Tom
Published: March 2017

For most of the 1590s, the Admiral's Men were the main competitors of Shakespeare's company in the London theatres. Not only did they stage old plays by dramatists such as Christopher Marlowe and Thomas Kyd: their playwrights invented the genres of humours comedy (with An Humorous Day's Mirth) and city comedy (with Englishmen for My Money), while other new plays such as A Knack to Know an Honest Man and The Downfall of Robert, Earl of Huntingdon were important influences on Shakespeare. This is the first book to read the Admiral's repertory against Shakespeare's plays of the 1590s, showing both how Shakespeare drew on their innovations and how his plays influenced Admiral's dramatists in turn. Shedding new light on well-known plays and offering detailed analysis of less familiar ones, it offers a fresh perspective on the dramatic culture of the 1590s.
Sandinista Nicaragua's Resistance to US Coercion
Authors: Perla, Jr Héctor
Published: March 2017

How was the Sandinista National Liberation Front (FSLN) of Nicaragua able to resist the Reagan Administration's coercive efforts to rollback their revolution? Héctor Perla challenges conventional understandings of this conflict by tracing the process through which Nicaraguans, both at home and in the diaspora, defeated US aggression in a highly unequal confrontation. He argues that beyond traditional diplomatic, military, and domestic state policies a crucial element of the FSLN's defensive strategy was the mobilization of a transnational social movement to build public opposition to Reagan's policy within the United States, thus preventing further escalation of the conflict. Using a contentious politics approach, the author reveals how the extant scholarly assumptions of international relations theory have obscured some of the most consequential dynamics of the case. This is a fascinating study illustrating how supposedly powerless actors were able to constrain the policies of the most powerful nation on earth.
Virgil's Ascanius
Authors: Rogerson Anne
Published: March 2017

Ascanius is the most prominent child hero in Virgil's Aeneid. He accompanies his father from Troy to Italy and is present from the first book of the epic to the last; he is destined to found the city of Alba Longa and the Julian family to which Caesar and Augustus both belonged; and he hunts, fights, makes speeches, and even makes a joke. In this first book-length study of Virgil's Ascanius, Anne Rogerson demonstrates the importance of this character not just to the Augustan family tree but to the texture and the meaning of the Aeneid. As a figure of prophecy and a symbol both of hopes for the future and of present uncertainties, Ascanius is a fusion of epic and dynastic desires. Compelling close readings of the representation and reception of this understudied character throughout the Aeneid expose the unexpectedly childish qualities of Virgil's heroic epic.
The International Responsibility of International Organisations
Authors: Moelle Moritz P.
Published: March 2017

The International Responsibility of International Organisations addresses the joint responsibility of organisations for violations of international law committed during the deployment of peacekeeping operations. More specifically, it inquires if and under which circumstances - in terms of the notion of control - international organisations can be jointly responsible. The author analyses the practice of international organisations (the United Nations, NATO, the European Union, the African Union and the Economic Community of West African States) on an inter-institutional level, as well as in the field in the form of five case studies. The likelihood and distribution of responsibility between international organisations engaged in peacekeeping operations is affected by the different layers of applicable primary norms (Security Council mandates, internal law of the organisations, international humanitarian and human rights law). Although external pressure may contribute to enhancing the effectiveness of holding international organisations jointly responsible, any substantial measures and mechanisms can only be implemented with the participation of states and international organisations.
Managerial Ties, Market Orientation, and Export Performance: Chinese Firms Experience
Authors: Yan Hui , He Xinming , Cheng Binwu
Published: February 2017

Managerial ties (MT) are important for business performance by providing firms access to valuable resources and protecting them from opportunism. Drawing on the resource-based view and the market orientation (MO) literature, we argue that (1) MT can help exporting firms to enhance export performance; and (2) MO will help strengthen the positive effect of MT as MO directs the value of MT for improvement of competitive strategy and customer experience with a market focus on generation, dissemination, and use of market intelligence concerning existing and potential customers and competitors. Using a sample of 230 Chinese exporting firms, we found that MT is linked to superior export performance, and the link is positively moderated by MO. Therefore, this study expands our understanding of how firms can not only improve their export performance through the development of MT, but also use MO to reinforce MT and export performance association.
A Leicestershire Recusant Family: The Nevills of Nevill Holt – I
Authors: Elliott Bernard
Published: February 2017

At the Reformation, three possibilities faced English Catholics. They could continue to be Catholics and so suffer the penalties of the penal laws; they could conform to the Church of England; or they could adopt a middle course and become Church Papists. The Nevills of Nevill Holt, near Market Harborough in Leicestershire, went through all three phases. In the reign of Edward VI, Thomas Nevill I became a Protestant. His grandson, Thomas Nevill II, became a Church Papist under James I; and Thomas II’s son, Henry Nevill I, continued to be one at the time of the Civil War. But Henry l’s son William was definitely a Catholic and went into exile with King James II, while William’s son, Henry Nevill II, was an open Catholic under Charles II. Henry Nevill II’s descendants continued to be Catholics throughout the eighteenth and nineteenth centuries until they left Nevill Holt in the late nineteenth century.
Robert Cornthwaite: a Neglected Nineteenth Century Bishop
Authors: Supple Jennifer F.
Published: February 2017

Several nineteenth-century Catholic bishops have found biographers, not only, as might be expected, the three archbishops of Westminster but also Ullathorne of Birmingham and Grant of South-wark. Yet Robert Cornthwaite, who was bishop in Yorkshire for almost twenty-nine years, laying the foundations for the three modern dioceses of Middlesbrough, Leeds and Hallam; who was Manning’s chief supporter at the Vatican Council and the most Ultramontane of the English bishops; and who was that allegedly rare creature, both an able administrator and a man of great spirituality, has been neglected. It is surely time to remedy matters.
The Birthplace and Parentage of Bishop Challoner: An Enquiry
Authors: Whatmore L. E.
Published: February 2017

The birth and parentage of Bishop Challoner have always been somewhat obscure. The first life by the Rev. James Barnard was published only three years after his death. But, though Barnard knew him for twenty years, his first chapter is disappointing. There are about fourteen pages on the marks of the Church and only about twelve lines on the early life of Challoner!
The Ripon Diary, 1878–80: III
Authors: Rossi John P.
Published: February 2017

Unlike parts one and two, the third section of the Ripon diary is almost solely concerned with political matters. Ripon's initial reluctance to assert himself politically, following his conversion, began to disappear as the prospect of a general election approached. He was now determined on a political career once again and as a result accepted numerous invitations to speak at Liberal party rallies in late 1879 and early 1880: so many that he began to feel that he might appear too ambitious. Toward the end of 1879 he noted in his diary that he would turn down an invitation to speak at a Liberal meeting in Leeds precisely because he had recently put himself forward so much. It is revealing of his renewed political ambition that Ripon ultimately changed his mind and went to Leeds.
The Return of the Cistercians to the Midlands
Authors: Elliott Bernard
Published: February 2017

It is sometimes stated that Mount St Bernard’s Abbey in the Charnwood Forest, Leicestershire, was the first Cistercian monastery to be established in England after the Reformation. But this is not so, for from 1794 to 1817, a Cistercian monastery flourished at Lulworth in Dorset, These monks, who came originally from La Trappe in France, were at the French Revolution expelled and found refuge in Switzerland. In 1794, Dom Augustine, the superior, determined to found a Cistercian house in Canada, and for that purpose several monks set out from Switzerland to Canada, travelling via England. On their arrival in London, however, they were persuaded by Thomas Weld of Lulworth Castle to remain in this country. Weld gave them a home in his park at Lulworth, where a monastery was shortly afterwards built and placed under the patronage of St Susan. Over the next few years these French Cistercians were joined by many English and Irish novices, but in 1817, they received a command from the British Government either to cease to receive British subjects or to leave the country. They chose the latter alternative and left England on 10 July 1817, over sixty in number, bound for Melleray in Brittany, where they took over the former monastery, still standing after the Revolution.
Midhurst Catholics and the Protestation Returns of 1642
Authors: McCann Timothy J.
Published: February 2017

Two recent articles inRecusant Historyhave drawn attention to the Protestation Returns, and used them as evidence for the size and strength of the Catholic community in the seventeenth century, as well as for evidence for the existence or non-existence of ‘Church Papists’. The purpose of this article is to move the discussion on towards an examination of the reliability of the Protestation Returns themselves as giving an accurate picture of the size of the Catholic community, and, in particular, to examine the evidence in the case of the parish of Midhurst in West Sussex, a well-known centre of recusancy.
Newsletter 1983
Authors: Davidson Alan
Published: February 2017

This bibliography, like those in previous Newsletters, has been compiled largely from a search of periodicals, especially those taken by the Institute of Historical Research. The items listed cover the period from the accession of Elizabeth I to the first Vatican Council. The publications of the Catholic Record Society, the Catholic Archives Society and the local recusant societies (Essex Recusant, Kent Recusant History, London Recusant, Northern Catholic History, North-West Catholic History, Staffordshire Catholic History and Worcestershire Recusant) have not been included.
Brough Hall, Catterick, and Thorpe Hall, Norwich: A Comment
Authors: Hodgetts Michael
Published: February 2017

There was a time when recusant history was written almost entirely from documents and architectural evidence was ignored, except by authors like Camm and Squiers. But at least it could be argued then that papers were more perishable than brick and stone, and in more urgent need of recording and preservation. Less than twenty years ago, in 1958, the theme of the first Oxford Conference on recusant history was ‘The Vanishing Archive’. Today, a much graver threat is that of the vanishing building. An exhibition held in London recently to mark European Architectural Heritage Year included records of at least twenty-five recusant houses that have been demolished, and in the last three months there have been two more cases which illustrate the urgency of the problem.

At the end of 1975, the Secretary for the Environment allowed the demolition of almost two-thirds of Brough Hall, near Catterick in Yorkshire. The central block of the house is Elizabethan and contains a priest-hole; about 1730 it was given a classical front, and forty years later two balancing wings were added by Thomas Atkinson. Now the east wing and all the centre except one pedimented bay are to be pulled down. This will mean the destruction of a panelled Elizabethan hall, of a Georgian room running the length of the first floor, of the hide, and of the Elizabethan friezes on the second floor. If, as often happens, the house must be reduced in size for economic reasons, the centre block should be preserved and the wings removed, as, for instance, at Mertoun in Berwickshire and (with the reluctant sacrifice of the chapel by William Ireland) at Houghton Hall in Yorkshire.
Forming capital: Emblematizing discourses of mobility in South Korea
Authors: Lo Adrienne , Choi Lee Jin
Published: March 2017

This article examines how listening subjects (Inoue 2006) mediate understandings of mobility in South Korea. Focusing on a cybercampaign to discredit the hip-hop star Tablo, it traces the ways that a listening public regimented signs relating to educational credentials, language, citizenship, and demeanor into forms of institutionalized, embodied, or objectified capital (Bourdieu 1986). Through the crafting of certain signs as icons that pointed to Tablo's ‘true’ character, and others as fronts, they were able to cast aspersions upon his command of English grammar, spelling, and interaction, and his character and educational credentials. By tracing how Tablo skeptics deployed metasemiotic discourses about emblems and figures of failed mobility, this article contributes to theories of semiosis that decenter the agentive speaking subject. (Capital, mobility, listening subject, English, South Korea)*
The ‘other woman’ in a mother and daughter relationship: The case of Mami Ji
Authors: Creese Angela , Blackledge Adrian
Published: February 2017

This article describes the range of discursive strategies in the socializing messages of a mother and daughter interaction. The analysis draws on the work of Bakhtin (1981) and Tannen (2007) to interrogate the role of a physically absent but discursively present sister-in-law, ‘Mami Ji’, across three speech events. Following Tannen, we show how the characterisation of the sister-in-law, Mami Ji, has chronotopic value that connects mother and daughter in the present and makes links across family histories. Through the discursive strategies of repetition, dialogue, detail, and translanguaging, ‘Mami Ji’ becomes an iconic benchmark of how not to speak, how not to dress, and how not to behave. Drawing on material from a linguistic ethnography approach, we present three discourse analyses from a much larger international project that also looked at classroom interaction and break-time conversations. The article contributes to the under-researched topic of the representation of sisters-in-law in discourse, theorises the chronotope in everyday conversation, and demonstrates how mother and daughter solidarity is achieved through opposition to another female family member. (Chronotope, linguistic involvement strategies, translanguaging, socialisation, sister-in-laws, mothers and daughters)
The Conversion of William Seymour, Duke of Somerset (1588–1660)
Authors: B. Fitzgibbon S.J.
Published: February 2017

William Seymour (1588–1660), the husband of the unfortunate Lady Arabella Stuart, first Marquis and second Earl of Hertford and second Duke of Somerset, was received into the Church at St Omer in the late months of 1611. He was the second son of Lord Beauchamp, (1561–1612), who inherited the Suffolk claim to the throne through his mother, Catherine Grey, (1538–1568), the sister of Lady Jane Grey, who according to the will of their grandfather, Henry VIII stood next in the succession to Elizabeth.
Some Catholic Musicians of the Eighteenth Century
Authors: Reynolds E. E.
Published: February 2017

During the second half of the eighteenth century there were a number of Catholic Musicians, both amateur and professional, who formed a friendly group of composers and performers. They met in each other's houses for concerts, and they joined together to raise the standard of music in the Embassy Chapels. They took an active part in such musical associations as the Madrigal Society, the Catch Club, and the Academy of Ancient Music. In this way they mixed with non-Catholics of like tastes.
“Popishe Thackwell” and Early Catholic Printing in Wales
Authors: Rogers D.M.
Published: February 2017

The distinction of putting into print the first book ever produced within the principality of Wales has recently been proved to belong to a group of Catholic outlaws operating in North Wales. Their book bears the date 1585 and though it was perhaps really printed in 1586 or early 1587, it antedates by over one hundred and thirty years the two ballads with which Isaac Carter inaugurated the modem press inside Wales at Trefhedyn in Cardiganshire in 1718 (1). The evidence for this press has gradually been pieced together over a number of years (2), but it is scattered in books and periodicals, and may here be brought together and compared with the bibliographical evidence of the book itself.
Postemergence Weed Control in Onion with Bentazon, Flumioxazin, and Oxyfluorfen
Authors: Herrmann Chad M. , Goll Margaret A. , Phillippo Colin J. , Zandstra Bernard H.
Published: February 2017

Field experiments were conducted in 2008 and 2010 to determine crop tolerance and weed control efficacy of the POST herbicides bentazon, flumioxazin, and oxyfluorfen applied to direct-seeded dry bulb onions on organic soil. Postemergence application of oxyfluorfen at 0.071 kg ai ha−1resulted in less than 20% onion injury when applied at the 2 and 4 onion leaf stages and provided good control of ladysthumb and common lambsquarters. Oxyfluorfen EC caused slightly higher visual injury than oxyfluorfen SC, but there was no difference in onion yield among the treatments. Application of flumioxazin at 0.036 of 0.072 kg ai ha−1alone or in combination with pendimethalin ACS resulted in minimal onion injury and no yield reduction. Combining flumioxazin in a tank mix with pendimethalin EC, dimethenamid-P EC, orS-metolachlor EC resulted in significant onion injury and yield reduction. Flumioxazin plusS-metolachlor, dimethenamid-P, or pendimethalin improved ladysthumb control in one of two years. Bentazon applied at 0.56 kg ai ha−1produced moderate onion injury and did not control yellow nutsedge adequately. Bentazon applied at 1.12 kg ai ha−1provided good control of yellow nutsedge but caused serious onion injury and yield loss.
Die Perspektive Angehöriger in der häuslichen Beatmungspflege
Authors: Lademann Julia , Schaepe Christiane , Ewers Michael
Published: March 2017

Zusammenfassung. Hintergrund: In Deutschland gewinnt die häusliche Versorgung beatmeter Patienten / innen an Bedeutung. Zur Situation darin involvierter Angehöriger gibt es aber kaum Daten. Ziel: Erkenntnisse zur Situation von Angehörigen in der häuslichen Beatmungspflege zu gewinnen, um ihre Rolle und Funktion in diesem Versorgungsbereich verstehen und einordnen zu können. Methode: Im Rahmen einer mehrteiligen qualitativen Studie wurden problemzentrierte Interviews mit Angehörigen (N = 15) von häuslich beatmeten Patienten / innen geführt und thematisch in Anlehnung an Prinzipien der Grounded Theory analysiert. Ergebnisse: Angehörige erbringen in der häuslichen Beatmungspflege ein unterschiedlich breites und anspruchsvolles Spektrum an Versorgungsleistungen (z. B. Haushalt, Organisation, pflegerisch-therapeutische Arbeit). Die Aufgaben- und Verantwortungsteilung zwischen den Pflegefachkräften und Angehörigen scheint aber in vielen Fällen ungeklärt und die Grenzen zwischen beider Expertise wenig reflektiert. Angehörige wünschen sich eine alltagsnahe Stärkung ihrer Kompetenzen durch qualifizierte Pflegefachkräfte – entsprechende Angebote (wenn es sie gibt) sind aus ihrer Sicht aber nicht immer hilfreich. Schlussfolgerungen: Angehörige, die sich in der häuslichen Beatmungspflege engagieren, sollten mit ihrem Wissen und Einsatz gewürdigt und nicht als Bittsteller und Dauerbelastete behandelt werden. Eine bedarfsgerechte professionelle Unterstützung müsste daher vor allem auf partnerschaftlichen Einbezug und gezielte Förderung ihrer Expertise setzen.