bluuebunny/crossref_metadata_2025 huggingface dataset:
Beals syndrome (congenital contractural arachnodactyly): prenatal ultrasound findings and molecular analysis
Authors: Inbar‐Feigenberg M. , Meirowitz N. , Nanda D. , Toi A. , Okun N. , Chitayat D.
Published: March 2014

We report the prenatal findings in two cases of Beals syndrome. Both pregnancies presented with clinical features of arthrogryposis multiplex congenita/fetal akinesia syndrome (AMC/FAS), including clenched fists and multiple joint contractures on repeat prenatal ultrasound examinations. The first case was diagnosed as having Beals syndrome on physical examination shortly after birth and the diagnosis was confirmed by DNA analysis, shown as a point mutation in the fibrillin 2 (FBN2) gene. The second case was diagnosed with Beals syndrome following microarray analysis on amniocytes, which showed a deletion of the FBN2 gene. Although most cases with AMC/FAS carry a poor prognosis, Beals syndrome is consistent with normal cognitive development and a better prognosis. Thus, making the correct diagnosis is crucial, both pre‐ and postnatally, for accurate counseling and management. Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd.
Hereditäre Optikusatrophien
Authors: Neuhann T.M. , Rautenstrauss B.
Published: March 2014

Bei hereditären Optikusatrophien werden 2 Untergruppen differenziert: isolierte hereditäre Optikusatrophien und hereditäre Optikusatrophien als Teil von syndromalen Erkrankungen. In der 1. Gruppe ist die Beeinträchtigung des N. opticus typischerweise die einzige Manifestation. Diese Gruppe umfasst insbesondere autosomal-dominante und autosomal-rezessive Optikusatrophien, darüber hinaus auch die mitochondrial vererbte hereditäre Leber-Optikusneuropathie (LHON).

In der 2. Gruppe, die die syndromalen Erkrankungen umfasst, wird eine Vielzahl neurologischer und anderer systemischer Auffälligkeiten beobachtet. Am häufigsten sind hier Veränderungen der mitochondrialen DNA (mtDNA) ursächlich. Weiterhin ist eine Optikusatrophie Symptom von einigen erblichen peripheren Neuropathien bzw. Charcot-Marie-Tooth-Erkrankungen (CMT2A2, CMTX5), hereditären sensorischen Neuropathie Typ 3 (HSAN3), Friedreich-Ataxie, Leukodystrophien, Sphingolipidosen, Zeroidlipofuszinosen und Eisenspeichererkrankungen („neurodegeneration with brain iron accumulation“, NBIA). Im vorliegenden Beitrag werden die zugrundeliegenden genetischen Prädispositionen und die klinischen Phänotypen erläutert.
Sharing stories of discrimination with parents
Authors: Juang Linda P. , Syed Moin
Published: March 2014

In this study we examined whether experiences of discrimination during childhood and adolescence were told to parents, the reasons for not telling, and whether telling was associated with adolescent characteristics and aspects of the current parent–child relationship. The sample included 200 ethnically diverse college students. Results supported a transactional view of socialization whereby youth who had not shared their discrimination stories expected negative parental reactions or were concerned for their parents' well‐being. The likelihood of telling was not directly associated with ethnicity, gender, or parent–child communication. Rather, those who rated the discrimination event as more important and reported greater current parental cultural and racial socialization were more likely to have disclosed their story of discrimination, depending on ethnicity. Understanding disclosure of discrimination experiences is important. When adolescents choose not to share experiences of discrimination with their parents, there may be lost opportunities for a greater understanding of these challenging experiences.
Risk factors for incident dementia after stroke and transient ischemic attack
Authors: Yang Jie , Wong Adrian , Wang Zhaolu , Liu Wenyan , Au Lisa , Xiong Yunyun , Chu Winnie W.C. , Leung Eric Y.L. , Chen Sirong , Lau Christine , Chan Anne Y.Y. , Lau Alexander Y.L. , Fan Florence , Ip Vincent , Soo Yannie , Leung Thomas , Ho Chi L. , Wong Lawrence K.S. , Mok Vincent C.T.
Published: March 2014

Background

We hypothesized that chronic brain changes are important substrates for incident dementia after stroke and transient ischemic attack (TIA).

Methods

We compared clinical and imaging features between patients with consecutive stroke/TIA with (n = 88) and without (n = 925) incident dementia at 3 to 6 months after a stroke/TIA. Pittsburg compound B (PiB) positron emission tomography was performed in 50 patients, including those with (n = 37) and without (n = 13) incident dementia.

Results

Age, history of diabetes mellitus, severity of white matter changes (WMCs), and medial temporal lobe atrophy (MTLA) were associated with incident dementia. Alzheimer's disease (AD)–like PiB retention was found in 29.7% and 7.7% (P = .032) of patients with and without incident dementia, respectively.

Conclusions

Chronic brain changes including WMCs, MTLA, and AD pathology are associated with incident dementia after stroke/TIA. Interventions targeting these chronic brain changes may reduce burden of vascular cognitive impairment.
Through A Japanese Prism: Foreign influence and Chinese telecommunications in the early Republican era
Authors: YANG DAQING
Published: March 2014

This paper examines the key modern infrastructure of telecommunications in early Republican China, through the eyes of a Japanese, Nakayama Ryûji, who served as a telecommunications adviser to the Chinese government from 1913 to 1928. Nakayama's numerous reports and recommendations to his Chinese employer and frequent confidential dispatches to the Japanese government, when read together, constitute a fascinating prism. They not only reveal problems as well as the potential in China's telecommunications sector, they also highlight Japan's efforts to compete with other foreign actors in China through the provision of Japanese equipment, expertise, and loans. While Nakayama strove to shape China's telecommunications development in ways that would, in his view, benefit both China and Japan, his efforts were often undercut by the aggressive actions of the Japanese government in China, such as the infamous Twenty-One Demands. Though promising at first, Japan's influence on China's modernization in the early Republican era came to be more limited, especially when compared with the final decade of the Qing Dynasty. Ultimately, what can be seen through this Japanese prism confirms that the development of an information infrastructure in modern China, as elsewhere, was as much shaped by technological and economic forces as it was influenced by political and diplomatic factors.
Embedding the symbolic dynamics of Lorenz maps
Authors: SAMUEL TONY , SNIGIREVA NINA , VINCE ANDREW
Published: March 2014

Necessary and sufficient conditions for the symbolic dynamics of a given Lorenz map to be fully embedded in the symbolic dynamics of a piecewise continuous interval map are given. As an application of this embedding result, we describe a new algorithm for calculating the topological entropy of a Lorenz map.
Homogeneous dispersion of high-conductive reduced graphene oxide sheets for polymethylmethacrylate nanocomposites
Authors: Zeng Xiaopeng , Yang Jingjing , Zhang Lizhen , Chen Lijuan , Yuan Wenxia
Published: March 2014

The high cohesive interaction between reduced graphene oxide (RGO) sheets usually makes them difficult to disperse, which limits their utilization in achieving effective hybridization with polymers. We report here a new two-step route for preparing non-aggregated and high-conductive RGO powders. Graphene oxide precursor was first reduced by hydrazine hydrate in presence of a thermal unstable surfactant of cetyltrimethylammonium chloride (CTAC). Then a thermal annealing process under H2/Ar atmosphere was further used to remove the non-conductive CTAC molecules. The prepared RGO powder exhibited an electrical conductivity of 2.23 × 104 S m−1 – about ten times higher than the one (N-RGO) simply reduced by hydrazine hydrate. After incorporating into polymethylmethacrylate with a 5 wt% loading, the composite showed a conductivity of 4.11 S m−1, which was 60 times as high as that of the same composite based on N-RGO powder. The addition and subsequent removal of CTAC molecules is an effective method for preparing non-aggregated and highly conductive graphene powder and obtaining good incorporation into polymer matrices.
Vitamin D in older people
Authors: Witham Miles D , Francis Gavin
Published: March 2014

Vitamin D has a wide range of biological effects beyond calcium and bone metabolism, and low 25-hydroxyvitamin D levels have been associated with many disease states in recent years, including cardiovascular disease, diabetes, infections and cancer. Association studies of vitamin D are notoriously prone to confounding and to reverse causality, however, and current intervention trial data for non-skeletal indications have been disappointing. Vitamin D is indicated for treatment of osteomalacia in older people, falls prevention in institutionalized older people, and as adjunctive therapy for osteoporosis. Large trials currently underway will ascertain whether potentially beneficial effects of vitamin D supplementation on all-cause mortality in older people are borne out.
Novel visualised three-dimensional images are useful for treating coronary artery fistulas in infants
Authors: Nakanishi Keisuke , Morita Terumasa , Amano Atsushi
Published: March 2014

Here we report an infant with coronary artery fistulas. To clearly visualise the fistulas and surrounding materials, we utilised three-dimensional computed tomography, and the images were transferred as novel visualised three-dimensional images using a reconstruction technique. With the images, we could repair the fistulas accurately. We believe that these images are useful for repairing coronary artery fistulas in infants.
A Paramedic Field Supervisor's Situational Awareness in Prehospital Emergency Care
Authors: Norri-Sederholm Teija , Kuusisto Rauno , Kurola Jouni , Saranto Kaija , Paakkonen Heikki
Published: March 2014

Introduction

Situational awareness (SA), or being aware of what is going on and what might happen next, is essential for the successful management of prehospital emergency care. However, far too little attention has been paid to the flow of information. Having the right information is important when formulating plans and actions.

Problem

The aim of this study was to analyze and describe the type of information that is meaningful for SA in the work of paramedic field supervisors, and to create an information profile for them in the context of prehospital emergency care.

Methods

Data were collected from January through March 2012 from semi-structured interviews with ten paramedic field supervisors representing four rescue departments in Finland. The interviews were based on three different types of real-life scenarios in the context of prehospital emergency care, and deductive content analysis was employed according to the information exchange meta-model. Data management and analysis were performed using Atlas.ti 7.

Results

A paramedic field supervisor information interest profile was formulated. The most important information categories were Events, Means, Action Patterns, and Decisions. The profile showed that paramedic field supervisors had four roles – situation follower, analyzer, planner and decision maker – and they acted in all four roles at the same time in the planning and execution phases.

Conclusion

Paramedic field supervisors are multitasking persons, building SA by using the available data, combining it with extensive know-how from their working methods and competencies, and their tacit knowledge. The results can be used in developing work processes, training programs, and information systems.

Norri-SederholmT,KuusistoR,KurolaJ,SarantoK,PaakkonenH.A paramedic field supervisor's situational awareness in prehospital emergency care.Prehosp Disaster Med.2014;29(2):1-9.
The Effects of Computerized Cognitive Control Training on Community Adults with Depressed Mood
Authors: Calkins Amanda W. , McMorran Katherine E. , Siegle Greg J. , Otto Michael W.
Published: March 2014

Background:Depression is frequently characterized by patterns of inflexible, maladaptive, and ruminative thinking styles, which are thought to result from a combination of decreased attentional control, decreased executive functioning, and increased negative affect. Cognitive Control Training (CCT) uses computer-based behavioral exercises with the aim of strengthening cognitive and emotional functions. A previous study found that severely depressed participants who received CCT exhibited reduced negative affect and rumination as well as improved concentration.Aims:The present study aimed to extend this line of research by employing a more stringent control group and testing the efficacy of three sessions of CCT over a 2-week period in a community population with depressed mood.Method:Forty-eight participants with high Beck Depression Inventory (BDI-II) scores were randomized to CCT or a comparison condition (Peripheral Vision Training; PVT).Results:Significant large effect sizes favoring CCT over PVT were found on the BDI-II (d= 0.73,p< .05) indicating CCT was effective in reducing negative mood. Additionally, correlations showed significant relationships between CCT performance (indicating ability to focus attention on CCT) and state affect ratings.Conclusions:Our results suggest that CCT is effective in altering depressed mood, although it may be specific to select mood dimensions.
INCOME INEQUALITY, MOBILITY, AND THE ACCUMULATION OF CAPITAL
Authors: García-Peñalosa Cecilia , Turnovsky Stephen J.
Published: March 2014

We examine the determinants of income mobility and inequality in a Ramsey model with elastic labor supply and heterogeneous wealth and ability. Both agents with lower wealth and those with greater ability tend to supply more labor, implying that labor supply decisions may have an equalizing or unequalizing effect depending on the relative importance of the two sources of heterogeneity. Moreover, these decisions are central to the extent of mobility observed in an economy. The relationship between mobility and inequality is complex. For example, a reduction in the interest rate and an increase in the wage rate reduce capital income inequality and allow upward mobility of the ability-rich. However, the increase in the labor supply of high-ability agents in response to higher wages raises earnings dispersion and thus has an offsetting effect. As a result, high mobility can be associated with an increase or a decrease in overall income inequality.
Comparing Factor, Class, and Mixture Models of Cannabis Initiation and DSM Cannabis Use Disorder Criteria, Including Craving, in the Brisbane Longitudinal Twin Study
Authors: Kubarych Thomas S. , Kendler Kenneth S. , Aggen Steven H. , Estabrook Ryne , Edwards Alexis C. , Clark Shaunna L. , Martin Nicholas G. , Hickie Ian B. , Neale Michael C. , Gillespie Nathan A.
Published: March 2014

Accumulating evidence suggests that the Diagnostic and Statistical Manual of Mental Disorders (DSM) diagnostic criteria for cannabis abuse and dependence are best represented by a single underlying factor. However, it remains possible that models with additional factors, or latent class models or hybrid models, may better explain the data. Using structured interviews, 626 adult male and female twins provided complete data on symptoms of cannabis abuse and dependence, plus a craving criterion. We compared latent factor analysis, latent class analysis, and factor mixture modeling using normal theory marginal maximum likelihood for ordinal data. Our aim was to derive a parsimonious, best-fitting cannabis use disorder (CUD) phenotype based on DSM-IV criteria and determine whether DSM-5 craving loads onto a general factor. When compared with latent class and mixture models, factor models provided a better fit to the data. When conditioned on initiation and cannabis use, the association between criteria for abuse, dependence, withdrawal, and craving were best explained by two correlated latent factors for males and females: a general risk factor to CUD and a factor capturing the symptoms of social and occupational impairment as a consequence of frequent use. Secondary analyses revealed a modest increase in the prevalence of DSM-5 CUD compared with DSM-IV cannabis abuse or dependence. It is concluded that, in addition to a general factor with loadings on cannabis use and symptoms of abuse, dependence, withdrawal, and craving, a second clinically relevant factor defined by features of social and occupational impairment was also found for frequent cannabis use.
Inhibition of post-translational N-glycosylation by HRD1 that controls the fate of ABCG5/8 transporter
Authors: Suzuki Shingo , Shuto Tsuyoshi , Sato Takashi , Kaneko Masayuki , Takada Tappei , Suico Mary Ann , Cyr Douglas M. , Suzuki Hiroshi , Kai Hirofumi
Published: March 2014

N-glycosylation of proteins in endoplasmic reticulum is critical for protein quality control. We showed here a post-translational N-glycosylation affected by the HRD1 E3 ubiquitin ligase. Both WT- and E3-defective C329S-HRD1 decreased the level of high mannose form of ABCG8, a protein that heterodimerizes with ABCG5 to control sterol balance. Meanwhile, HRD1 increased the non-glycosylated ABCG8 regardless of its E3 activity, thereby suppressing full maturation of ABCG5/8 transporter. Pulse chase and mutational analysis indicated that HRD1 inhibits STT3B-dependent post-translational N-glycosylation of ABCG8. Whereas, HRD1 had only slight effect on the N-glycosylation status of ABCG5; rather it accelerated ABCG5 degradation in an E3 activity-dependent manner. Finally, RMA1, another E3 ubiquitin ligase, accelerated the degradation of both ABCG5 and ABCG8 via E3 activity-dependent manner. HRD1 and RMA1 may therefore be negative regulators of disease-associated transporter ABCG5/ABCG8. The findings also highlight the unexpected E3 activity-independent role of HRD1 in the regulation of N-glycosylation.
Polymeric micelles with π–π conjugated moiety on glycerol dendrimer as lipophilic segments for anticancer drug delivery
Authors: Li Yuanlin , Su Ting , Li Sai , Lai Yusi , He Bin , Gu Zhongwei
Published: March 2014

Novel polymeric micelles with cinnamate moieties immobilized on glycerol dendrimer as lipophilic segments were reported as carriers for drug delivery.
Rh-Catalyzed oxidative C–H activation/annulation: converting anilines to indoles using molecular oxygen as the sole oxidant
Authors: Zhang Guoying , Yu Hui , Qin Guiping , Huang Hanmin
Published: March 2014

A practical and efficient Rh(iii)-catalyzed oxidative C–H annulation using molecular oxygen as the sole oxidant has been developed for the facile synthesis of a broad range of indoles from simple anilines and alkynes.
Mesoporous 3D ZnO–NiO architectures for high-performance supercapacitor electrode materials
Authors: Wei Chengzhen , Pang Huan , Cheng Cheng , Zhao Junhong , Li Pengwei , Zhang Yongkang
Published: March 2014

3D ZnO–NiO mesoporous architectures were synthesized through annealing the zinc hydroxide carbonate–nickel hydroxide carbonate composite precursor, which was prepared via a one-pot hydrothermal route.
Competition between hydrogen bonding and dispersion interactions in the crystal structures of the primary amines
Authors: Maloney Andrew G. P. , Wood Peter A. , Parsons Simon
Published: March 2014

In the short chain amines H-bonding dominates crystal packing, but dispersion wins-out for the long chain compounds. The cross-over point occurs between butyl and pentylamine, where interactions are finely balanced.
Defect reduction in semipolar {101̄3̄} GaN grown on m-sapphire via two-step nanoepitaxial lateral overgrowth
Authors: Yang Jiankun , Wei Tongbo , Huo Ziqiang , Zhang Yonghui , Hu Qiang , Wei Xuecheng , Sun Baojuan , Duan Ruifei , Wang Junxi
Published: March 2014

A method to obtain high quality semipolar {101̄3̄} GaN grown on m-plane sapphire is presented. This method is similar to two-step nanoepitaxial lateral overgrowth (2S-NELOG) by combining a TiN interlayer and self-assembled SiO2 nanospheres.
Static 1H dynamic nuclear polarization with the biradical TOTAPOL: a transition between the solid effect and the cross effect
Authors: Shimon Daphna , Feintuch Akiva , Goldfarb Daniella , Vega Shimon
Published: March 2014

Static 1H-DNP with TOTAPOL: the solid effect (SE) dominant at low temperatures; the cross effect (CE) dominant at high temperatures; and DNP-buildup: Tbu(SE) < Tbu(CE).
Mass spectrometric investigation of the role of the linking polypeptide chain in DNA polymerase I
Authors: Yeom Taeho , Lee Jungyoon , Lee Seonghyun , Kang Sunah , Kim Kyung Rok , Han Byungwoo , Lee Hyun Soo , Jo Kyubong
Published: March 2014

MALDI-TOF analysis elucidates the functions of two domains in pol I.
A coumarin-based fluorescent probe for recognition of Cu2+and fast detection of histidine in hard-to-transfect cells by a sensing ensemble approach
Authors: You Qi-Hua , Lee Albert Wai-Ming , Chan Wing-Hong , Zhu Xiao-Ming , Leung Ken Cham-Fai
Published: March 2014

A Cu2+responsive fluorescent chemosensorCAQAwas used to prepare a sensing ensemble for detecting histidine in hard-to-transfect cells.
Arene non-innocence in dinuclear complexes of Fe, Co, and Ni supported by a para-terphenyl diphosphine
Authors: Horak Kyle T. , Velian Alexandra , Day Michael W. , Agapie Theodor
Published: March 2014

Hapticity changes of a Fe2(μ-arene) moiety upon one-electron oxidation highlight the use of an arene as a non-innocent ligand.
Size and ability do matter! Influence of acidity and pore size on the synthesis of hindered halogenated meso-phenyl porphyrins catalysed by porous solid oxides
Authors: Silva Mónica , Fernandes Auguste , Bebiano Suse S. , Calvete Mário J. F. , Ribeiro M. Filipa , Burrows Hugh D. , Pereira Mariette M.
Published: March 2014

Rationalisation of acidity and pore size influence on synthesis of hindered mesoarylporphyrins. Efficient, reusable and scalable alternative for one-pot synthesis in high yields.
n-Bu4NI-catalyzed selective dual amination of sp3 C–H bonds: oxidative domino synthesis of imidazo[1,5-c]quinazolines on a gram-scale
Authors: Zhao Dan , Wang Teng , Shen Qi , Li Jian-Xin
Published: March 2014

A facile and efficient approach to the synthesis of imidazo[1,5-c]quinazolines was developed via a tandem reaction following dual sp3 C–H amination under metal-free conditions.
Drug solid solutions – a method for tuning phase transformations
Authors: Delori Amit , Maclure Pauline , Bhardwaj Rajni M. , Johnston Andrea , Florence Alastair J. , Sutcliffe Oliver B. , Oswald Iain D. H.
Published: March 2014

Tuning phase transformation temperatures through the use of solid solutions.
Hydrothermal synthesis of single-crystalline tetragonal perovskite PbTiO3 nanosheets with dominant (001) or (111) facets
Authors: Xu Gang , Huang Xiaoqiang , Krstic Vladimir , Chen Shuquan , Yang Xin , Chao Chunying , Shen Ge , Han Gaorong
Published: March 2014

Due to the difference in electronegativity, Na+ ions fasten on (001) planes, whereas K+ ions fasten on (111) planes, resulting in PbTiO3 nanosheets with dominant (001) or (111) facets, respectively.
Accurate calculations of geometries and singlet–triplet energy differences for active-site models of [NiFe] hydrogenase
Authors: Delcey Mickaël G. , Pierloot Kristine , Phung Quan M. , Vancoillie Steven , Lindh Roland , Ryde Ulf
Published: March 2014

The relative stability of singlet and triplet state models of [NiFe] hydrogenase have been studied by advanced quantum-mechanical methods.
Encapsulation of sodium alkyl sulfates by the cyclotriveratrylene-based, [Pd6L8]12+ stella octangula cage
Authors: Cookson Nikki J. , Henkelis James J. , Ansell Richard J. , Fishwick Colin W. G. , Hardie Michaele J. , Fisher Julie
Published: March 2014

1H NMR studies have revealed that a [Pd6L8]12+ stella octangula cage can act as host to two molecules of alkyl sulfate; with chain lengths of 8–14 carbons.
Recruiting general practitioners for surveys: reflections on the difficulties and some lessons learned
Authors: Parkinson Anne , Jorm Louisa , Douglas Kirsty A. , Gee Alison , Sargent Ginny M. , Lujic Sanja , McRae Ian S.
Published: March 2014

Surveys of GPs are essential to facilitate future planning and delivery of health services. However, recruitment of GPs into research has been disappointing with response rates declining over recent years. This study identified factors that facilitated or hampered GP recruitment in a recent survey of Australian GPs where a range of strategies were used to improve recruitment following poor initial responses. GP response rates for different stages of the survey were examined and compared with reasons GPs and leaders of university research networks cited for non-participation. Poor initial response rates were improved by including a questionnaire in the mail-out, changing the mail-out source from an unknown research team to locally known network leaders, approaching a group of GPs known to have research and training interests, and offering financial compensation. Response rates increased from below 1% for the first wave to 14.5% in the final wave. Using a known and trusted network of professionals to endorse the survey combined with an explicit compensation payment significantly enhanced GP response rates. To obtain response rates for surveys of GPs that are high enough to sustain external validity requires an approach that persuades GPs and their gatekeepers that it is worth their time to participate.
Conflicts and negotiations about framings of gender equality and diversity by political actors within the European public sphere
Authors: Siim Birte
Published: March 2014

This article addresses conflicts and negotiations about gender equality and diversity within the European public sphere (EPS). It aims to explore the specific constraints and possibilities for realizing gender equality and justice from the transnational European context. Research illustrates that the European Union (EU) approach to gender equality has strengthened the transnational space of mobilization and given actors new possibilities to negotiate about multiple inequalities and direct their claims at different levels. This has at the same time led to a diversification of women's claims and conflicts about the meanings and framings of gender equality and diversity within the EPS. This is illustrated by case studies of the framing of gender equality and ethno-national diversity by political actors in the European Women's Lobby and the European Parliament. The article proposes that the multilevel EU polity can be interpreted as a unique case for reframing justice in a globalized world.
Preperoxisomal vesicles can form in the absence of Pex3
Authors: Knoops Kèvin , Manivannan Selvambigai , Cepińska Małgorzata N. , Krikken Arjen M. , Kram Anita M. , Veenhuis Marten , van der Klei Ida J.
Published: March 2014

We demonstrate that the peroxin Pex3 is not required for the formation of peroxisomal membrane structures in yeast pex3 mutant cells. Notably, pex3 mutant cells already contain reticular and vesicular structures that harbor key proteins of the peroxisomal receptor docking complex—Pex13 and Pex14—as well as the matrix proteins Pex8 and alcohol oxidase. Other peroxisomal membrane proteins in these cells are unstable and transiently localized to the cytosol (Pex10, Pmp47) or endoplasmic reticulum (Pex11). These reticular and vesicular structures are more abundant in cells of a pex3 atg1 double deletion strain, as the absence of Pex3 may render them susceptible to autophagic degradation, which is blocked in this double mutant. Contrary to earlier suggestions, peroxisomes are not formed de novo from the endoplasmic reticulum when the PEX3 gene is reintroduced in pex3 cells. Instead, we find that reintroduced Pex3 sorts to the preperoxisomal structures in pex3 cells, after which these structures mature into normal peroxisomes.
Feroz Papa: Saving cells from themselves
Authors: Sedwick Caitlin
Published: March 2014

Papa studies adaptive and maladaptive responses to ER stress.
Management of Duodenal–Jejunal Polyps in Children With Peutz‐Jeghers Syndrome With Single‐Balloon Enteroscopy
Authors: Bizzarri Barbara , Borrelli Osvaldo , de'Angelis Nicola , Ghiselli Alessia , Nervi Giorgio , Manfredi Marco , de'Angelis Gian Luigi
Published: March 2014

Objectives:

Children with Peutz‐Jeghers syndrome (PJS) have increased risk of polyp‐related complications and emergency laparotomies. The aim of the present study was to assess the efficacy and the safety of endoscopic therapy of small bowel polyps using single‐balloon enteroscopy (SBE) in children affected by PJS.

Methods:

Between January 2010 and December 2011, prospectively consecutive PJS children with polyps >15 mm or polyps actively bleeding previously identified using video capsule endoscopy and magnetic resonance imaging underwent therapeutic SBE. The main outcome measurements were the feasibility, the technical performance, and the safety.

Results:

A total of 10 children (6 boys; median age 13.7 years, range 5.6–15.6) underwent 23 SBE procedures. Four patients had a history of abdominal surgery. A total of 53 polyps were removed, and 23 of them were >15 mm. The majority of polyps were found in jejunum (85%). The mean insertion depths for antegrade and retrograde approach were 200 ± 80 and 100 ± 50 cm beyond the ileal valve, respectively. The mean procedure time was 75 ± 25 minutes. Mild abdominal pain was reported after 3 procedures. In 1 patient a postpolypectomy perforation occurred.

Conclusions:

In conclusion, SBE is an effective endoscopic tool for treating small bowel polyps in children with PJS, and well‐timed polypectomy may optimize patients' care, preventing polyp‐related complications and emergency laparotomy. Further larger multicenter studies are warranted to accurately determine the safety of therapeutic SBE in children.
Topical Mitomycin‐C Application in Recurrent Esophageal Strictures After Surgical Repair of Esophageal Atresia
Authors: Chapuy Laurence , Pomerleau Martine , Faure Christophe
Published: March 2014

Objectives:

The aim of the present study was to evaluate the efficacy and short‐term safety of topical mitomycin‐C, an antifibrotic agent, in preventing the recurrence of anastomotic strictures after surgical repair of esophageal atresia (EA).

Methods:

We retrospectively reviewed the medical records of patients with recurrent anastomotic strictures after EA surgery who underwent at least 3 esophageal dilations. We compared the outcome (ie, resolution of the stricture) of the group that received topical mitomycin‐C treatment with endoscopic esophageal dilation with a historical cohort treated by dilations alone.

Results:

A total of 11 children received mitomycin‐C concurrently with endoscopic dilations. After a median follow‐up of 33 months (range 18–72), and a mean number of 5.4 dilations per patient (range 3–11), 8 of 11 patients achieved a resolution of their strictures, 2 patients remained with stenosis, and 1 patient needed a surgical correction. In the control group, 10 patients required an average of 3.7 (range 3–7) total dilations. After a follow‐up of 125 months (range 35–266) after the last dilation, strictures in 9 of 10 children disappeared and the remaining patient was symptom free. No dysplasia related to mitomycin‐C was demonstrated.

Conclusions:

There is no benefit in the resolution of the stricture when adding mitomycin‐C treatment compared with repeated esophageal dilations alone in historical controls. Further randomized controlled studies and a short‐ and long‐term evaluation of safety are needed.
Structural basis for promoter specificity switching of RNA polymerase by a phage factor
Authors: Tagami Shunsuke , Sekine Shun-ichi , Minakhin Leonid , Esyunina Daria , Akasaka Ryogo , Shirouzu Mikako , Kulbachinskiy Andrey , Severinov Konstantin , Yokoyama Shigeyuki
Published: March 2014

Transcription of DNA to RNA by DNA-dependent RNA polymerase (RNAP) is the first step of gene expression and a major regulation point. Bacteriophages hijack their host's transcription machinery and direct it to serve their needs. The gp39 protein encoded by Thermus thermophilus phage P23-45 binds the host's RNAP and inhibits transcription initiation from its major “−10/−35” class promoters. Phage promoters belonging to the minor “extended −10” class are minimally inhibited. We report the crystal structure of the T. thermophilus RNAP holoenzyme complexed with gp39, which explains the mechanism for RNAP promoter specificity switching. gp39 simultaneously binds to the RNAP β-flap domain and the C-terminal domain of the σ subunit (region 4 of the σ subunit [σ4]), thus relocating the β-flap tip and σ4. The ∼45 Å displacement of σ4 is incompatible with its binding to the −35 promoter consensus element, thus accounting for the inhibition of transcription from −10/−35 class promoters. In contrast, this conformational change is compatible with the recognition of extended −10 class promoters. These results provide the structural bases for the conformational modulation of the host's RNAP promoter specificity to switch gene expression toward supporting phage development for gp39 and, potentially, other phage proteins, such as T4 AsiA.
Small but sturdy: small RNAs in cellular memory and epigenetics
Authors: Stuwe Evelyn , Tóth Katalin Fejes , Aravin Alexei A.
Published: March 2014

Cells in multicellular organisms have distinct identities characterized by their profiles of expressed genes. Cell identities can be stable over a long time and through multiple cellular divisions but are also responsive to extracellular signals. Since the DNA sequence is identical in all cells, a “cellular memory” of expression profiles is achieved by what are defined as epigenetic mechanisms. Two major molecular principles—networks of transcription factors and maintenance of cis-chromatin modifications—have been implicated in maintaining cellular memory. Here we describe recent studies demonstrating that short noncoding RNAs can also provide molecular signals that define epigenetic states of cells. Small RNAs can act independently or cooperate with chromatin modifications to achieve long-lasting effects necessary for cellular memory and transgenerational inheritance.
Measurement of Intracellular Ca2+ Release in Permeabilized Cells Using 45Ca2+
Authors: Luyten Tomas , Bultynck Geert , Parys Jan B. , De Smedt Humbert , Missiaen Ludwig
Published: March 2014

This protocol describes a technique to measure Ca2+ release from the nonmitochondrial intracellular Ca2+ stores in monolayers of saponin-permeabilized cells cultured in 12-well 4-cm2 clusters. The 45Ca2+-flux technique described here can only be applied to cell types that still adhere to the plastic after exposing them to saponin. We describe the permeabilization procedure, the loading of the nonmitochondrial Ca2+ stores with 45Ca2+, and the subsequent 45Ca2+ efflux.
In Vivo Delivery of Lenti-Cre or Adeno-Cre into Mice Using Intranasal Instillation
Authors: Gierut Jessica J. , Jacks Tyler E. , Haigis Kevin M.
Published: March 2014

Lung cancer remains the leading cause of cancer deaths among both men and women, with a lower rate of survival than both breast and prostate cancer. Development of the Cre/lox system and improved mouse models have allowed researchers to gain a better understanding of human disease, including lung cancer. Through the viral delivery of Cre, gene function in adult mice can be precisely studied at a specific developmental stage or in a specific cell/tissue type of choice. This protocol describes how to produce adenovirus-Cre precipitate. Using this adeno-Cre (or lentivirus-Cre), Cre can be expressed in mouse lungs. The virus is delivered by intranasal instillation.
Visualizing Apoptosis in Embryos and the Germline of Caenorhabditis elegans
Authors: Lant Benjamin , Derry W. Brent
Published: March 2014

Visualizing apoptosis in developing embryos or the germline of Caenorhabditis elegans is remarkably easy because of the transparency of the organism. The invariant pattern of cell division and programmed cell death during development makes it possible to quantify small but reproducible changes in apoptosis, which are easy to detect by light microscopy because of the refractile properties of dying cells. Although apoptotic death is easy to visualize and quantify in the germline of adult hermaphrodites, the pattern of cell death is variable, especially when triggered by stress. The most convenient method for visualization of apoptosis in vivo is light microscopy, which requires immobilizing live embryos or adult animals on slides. This protocol describes the basic methods for visualizing and analyzing apoptosis in living animals.
Simultaneous Patch-Clamping and Calcium Imaging in Developing Dendrites
Authors: Kleindienst Thomas , Lohmann Christian
Published: March 2014

Calcium imaging has been used extensively to explore the role of action potential (AP) firing in the development of neuronal structure and synaptic function because increases in intracellular calcium ([Ca2+]i) reliably and, within a certain range, linearly reflect neuronal spiking activity. Patterns of APs in individual cells can be deduced from calcium recordings, which have typically been performed at the level of cell bodies. However, neurons are particularly susceptible to phototoxicity when they are illuminated at the soma. Furthermore, for some imaging experiments (e.g., those that address the interactions between dendrites and axons during synapse formation), the cell body of a given neuron may simply not be in the field of view. In these situations, it would be helpful to determine the spiking patterns of a neuron from the calcium activity in its subcellular compartments such as stretches of dendrites or axons. Here, we describe an approach for determining the relationship between AP firing and dendritic calcium transients by simultaneously imaging calcium transients in small dendritic stretches of hippocampal pyramidal neurons in slice cultures from neonatal rats and recording spiking activity with whole-cell patch-clamp recordings in these neurons. These experiments allow us to correlate the electrophysiological spiking pattern with the accompanying changes in the calcium concentration in individual dendritic segments.
Preparation of Ribonucleoprotein-Enriched Nucleoplasmic Extracts from Drosophila Cells
Authors: Rio Donald C.
Published: March 2014

Here we describe how to prepare nucleoplasmic extracts from suspension cultures of Drosophila S2 (Schneider Line 2) cells. Harvested cells are washed in phosphate-buffered saline supplemented with MgCl2, resuspended in hypotonic buffer, and homogenized. Nuclei are isolated by centrifugation and then sonicated. The nuclear sonicate is placed on a 30% sucrose cushion and sedimented. The soluble nuclear ribonucleoprotein (RNP) complexes remain in the supernatant and the nuclear membrane fragments and chromatin pellet through the sucrose cushion.
Measurement of Intracellular Ca2+ Release in Intact and Permeabilized Cells Using 45Ca2+
Authors: Missiaen Ludwig , Luyten Tomas , Bultynck Geert , Parys Jan B. , De Smedt Humbert
Published: March 2014

Ca2+ is an important ion that controls almost every function in a cell. Activator Ca2+ can be released from intracellular Ca2+ stores, and there are various ways to study this release. Here, we introduce a technique that uses radioactive 45Ca2+ to quantitatively measure the unidirectional release of Ca2+ from the nonmitochondrial Ca2+ stores in monolayers of cultured cells. This technique can be used in cells with an intact plasma membrane as well as in cells in which the plasma membrane has been permeabilized.
The marine diversity spectrum
Authors: Reuman Daniel C. , Gislason Henrik , Barnes Carolyn , Mélin Frédéric , Jennings Simon
Published: March 2014

Distributions of species body sizes within a taxonomic group, for example, mammals, are widely studied and important because they help illuminate the evolutionary processes that produced these distributions. Distributions of the sizes of species within an assemblage delineated by geography instead of taxonomy (all the species in a region regardless of clade) are much less studied but are equally important and will illuminate a different set of ecological and evolutionary processes.

We develop and test a mechanistic model of how diversity varies with body mass in marine ecosystems. The model predicts the form of the ‘diversity spectrum’, which quantifies the distribution of species' asymptotic body masses, is a species analogue of the classic size spectrum of individuals, and which we have found to be a new and widely applicable description of diversity patterns.

The marine diversity spectrum is predicted to be approximately linear across an asymptotic mass range spanning seven orders of magnitude. Slope −0·5 is predicted for the global marine diversity spectrum for all combined pelagic zones of continental shelf seas, and slopes for large regions are predicted to lie between −0·5 and −0·1. Slopes of −0·5 and −0·1 represent markedly different communities: a slope of −0·5 depicts a 10‐fold reduction in diversity for every 100‐fold increase in asymptotic mass; a slope of −0·1 depicts a 1·6‐fold reduction. Steeper slopes are predicted for larger or colder regions, meaning fewer large species per small species for such regions.

Predictions were largely validated by a global empirical analysis.

Results explain for the first time a new and widespread phenomenon of biodiversity. Results have implications for estimating numbers of species of small asymptotic mass, where taxonomic inventories are far from complete. Results show that the relationship between diversity and body mass can be explained from the dependence of predation behaviour, dispersal, and life history on body mass, and a neutral assumption about speciation and extinction.
The differential response of the Pben promoter of Pseudomonas putida mt‐2 to BenR and XylS prevents metabolic conflicts in m‐xylene biodegradation
Authors: Pérez‐Pantoja Danilo , Kim Juhyun , Silva‐Rocha Rafael , de Lorenzo Víctor
Published: March 2014

Pseudomonas putida mt‐2 encompasses two alternative and potentially conflicting routes for benzoate metabolism, one meta pathway encoded by xyl genes of the pWW0 plasmid and mastered by the Pm promoter and XylS, and one chromosomally encoded ortho pathway initiated by Pben and the BenR protein. Any cross‐activation of Pben promoter by XylS ought to cause a metabolic conflict during the degradation of m‐xylene because 3‐methylbenzoate (3MBz) generated as an intermediate can be channelled through the ortho pathway and produce toxic dead‐end metabolites. The activation of Pben by XylS was revisited using both reporter technology and tiling arrays targeted to the sequences of interest around messenger RNA initiation of both Pben and Pm promoters. Analysis of supersensitive luxCDABE fusions, inspection of xylX versus benA transcripts and growth tests of benR mutants indicated that the natural expression ranges of XylS under various conditions are insufficient to cause a significant cross‐regulation of Pben whether cells face endogenous or exogenous 3MBz. This seems to stem from the nature of the operators for binding either transcriptional factor, which in the case of the Pben promoter of P. putida mt‐2 appear to have evolved for avoiding a strong interaction with XylS.
Marine bacterioplankton community turnover within seasonally hypoxic waters of a subtropical sound: Devil's Hole, Bermuda
Authors: Parsons Rachel J. , Nelson Craig E. , Carlson Craig A. , Denman Carmen C. , Andersson Andreas J. , Kledzik Andrew L. , Vergin Kevin L. , McNally Sean P. , Treusch Alexander H. , Giovannoni Stephen J.
Published: March 2014

Understanding bacterioplankton community dynamics in coastal hypoxic environments is relevant to global biogeochemistry because coastal hypoxia is increasing worldwide. The temporal dynamics of bacterioplankton communities were analysed throughout the illuminated water column of Devil's Hole, Bermuda during the 6‐week annual transition from a strongly stratified water column with suboxic and high‐pCO2 bottom waters to a fully mixed and ventilated state during 2008. A suite of culture‐independent methods provided a quantitative spatiotemporal characterization of bacterioplankton community changes, including both direct counts and rRNA gene sequencing. During stratification, the surface waters were dominated by the SAR11 clade of Alphaproteobacteria and the cyanobacterium Synechococcus. In the suboxic bottom waters, cells from the order Chlorobiales prevailed, with gene sequences indicating members of the genera Chlorobium and Prosthecochloris – anoxygenic photoautotrophs that utilize sulfide as a source of electrons for photosynthesis. Transitional zones of hypoxia also exhibited elevated levels of methane‐ and sulfur‐oxidizing bacteria relative to the overlying waters. The abundance of both Thaumarcheota and Euryarcheota were elevated in the suboxic bottom waters (> 109 cells l−1). Following convective mixing, the entire water column returned to a community typical of oxygenated waters, with Euryarcheota only averaging 5% of cells, and Chlorobiales and Thaumarcheota absent.
Recasting Competition Concurrency under the Enterprise and Regulatory Reform Act 2013
Authors: Dunne Niamh
Published: March 2014

The concurrent enforcement power granted to certain sector economic regulators is one of the more remarkable features of UK competition law. In practice, regulators have tended to under‐enforce their competition powers, preferring to resolve market difficulties through regulatory interventions. Recent amendments to the concurrency framework, introduced by sections 51 to 53 of the Enterprise and Regulatory Reform Act 2013, seek both to strengthen the priority of competition enforcement and to provide plausible sanctions – including, ultimately, the removal of competition jurisdiction from regulators – for continued underuse. This article assesses these reforms in light of the history and (limited) application of the concurrent competition powers of regulators to date. It argues that the absence of an overarching policy rationale for this curious example of UK antitrust ‘exceptionalism’ complicates the determination of whether the reforms, which ostensibly seek to reinforce but potentially also undermine concurrency, are likely to have a positive market impact in practice.
Inhuman and Degrading Punishment, Dignity, and the Limits of Retribution
Authors: Mavronicola Natasa
Published: March 2014

The recent judgment of the Grand Chamber of the European Court of Human Rights (ECtHR) in Vinter and others v United Kingdom provides a much needed clarification of the parameters of the prohibition on inhuman and degrading punishment under Article 3 of the European Convention on Human Rights (ECHR) as it applies to whole life orders of imprisonment under mandatory life sentences – essentially, life imprisonment without parole. The Grand Chamber's judgment refines Strasbourg doctrine on life imprisonment and the prospect of release and illuminates key principles concerning inhuman and degrading punishment under Article 3 of the ECHR. This article considers the judgment's profound significance in relation to both human rights and penology.