bluuebunny/crossref_metadata_2025 huggingface dataset:
Conservation Theory and Practice: Materials, Values, and People in Heritage Conservation
Authors: Sully Dean
Published: December 2013

Conservation, as an emerging discipline, is mutually constituted with the heritage institutions in which it is practiced. Professional conservators have been amending the focus of their work, from attending to the material preservation of heritage spaces, places, and objects, toward the values that people have for their cultural heritage. This chapter describes an alternative conservation product, which is entangled within the multiple interactions between objects and people developed during the conservation event. This reflects a shift in focus from the materials from which conservation objects are composed, to the aspirations of the people who are affected by the conservation of their heritage. This challenges conventional heritage conservation practice, by privileging a community's cultural systems over universalized concepts of heritage. As a result, conservation practice requires principles, policies, and guidelines that help conservators to engage people in decision‐making about their heritage. New frameworks for understanding conservation practice enable creative and diverse solutions within heritage conservation. This is described in this chapter in terms of materials‐, values‐, and peoples‐based approach to conservation. This allows heritage conservation to address the social issues of the present and engage the future, rather than merely seeking to fix the past. In so doing, it validates conservation responses that seek to incorporate the multiple ways that people care for and use their own cultural heritage.
Developing Audiences for the Twenty‐First‐Century Museum
Authors: Black Graham
Published: December 2013

This chapter explores the challenges museums face in remaining relevant to twenty‐first‐century audiences in what is a period of rapid societal upheaval brought on by demographic change, generational shift, and the impact of new media. It explores the development of museum practice toward both traditional and new audiences and emphasizes that there is not a choice to be made between the two – museums need both. The author explores two case studies, the National Trust in the UK and the Dallas Museum of Art in the USA, which demonstrate how learning and visitor profiling can focus the work of audience development. Arguing that museums need to get to know their audiences much better and that the shifting expectations of audiences must lead to equal shift in the nature of the museum experience, it concludes that such change requires planning for the long term and sustained commitment.
What, or Where, Is the (Museum) Object?: Colonial Encounters in Displayed Worlds of Things
Authors: Dudley Sandra H.
Published: December 2013

This chapter considers an approach to conceptualizing and theorizing the moments, however brief they may be, in which museum visitors stop to look closely at things on display. Situating the chapter as part of a wider attempt to move away from representation as the starting point of museum analysis, I explore ways in which the engagements between people and things might be imagined from the object's point of view. In particular, I position the object–visitor interaction as analogous to the colonial encounter. I develop the colonial metaphor in order to reflect on the relative position of the two participants, examining the nature of the gaze and the object's capacity to gaze back (metaphorically at least). Once within this new, metaphorically colonial world, encounters between persons and things are different than they would be elsewhere, and objects and engagements with them have the potential to excite, inspire, disorder, or frighten. I then explore the object's perspective in more detail, positively appropriating the termprosopopoeia(the application of human characteristics to material objects, animals, or dead bodies). The chapter concludes that the approach advanced here can allow the development of both innovative material culture theory and fresh approaches to museum and gallery practice.
The Blackfoot Shirts Project: “Our Ancestors Have Come to Visit”
Authors: Brown Alison K. , Peers Laura
Published: December 2013

In 2010, at the request of Blackfoot ceremonial leaders, five early nineteenth century shirts in the collection of the Pitt Rivers Museum, University of Oxford, were loaned to museums in Southern Alberta, Canada, as part of a project aimed at exploring the contemporary meanings of these historic garments to Blackfoot people today. Before the shirts were exhibited, handling sessions – or “visits” – were arranged for Blackfoot elders, ceremonial leaders, artists, teachers, and high school and college students. These sessions provided opportunities for people to reconnect with the historic and cultural knowledge embedded within the shirts, much of which has become fragmented within their own communities. Interviews with session participants highlighted how such direct engagements with the shirts have contributed to the revitalization of artistic knowledge and ceremonial traditions. Such projects raise challenges, however, for both the museums and the communities engaged in them. This chapter explores the immediate impacts of the shirts’ visit to Blackfoot territory, and the tensions and difficulties raised by such work.
Reanimating Cultural Heritage: Digital Curatorship, Knowledge Networks, and Social Transformation in Sierra Leone
Authors: Basu Paul
Published: December 2013

The institution of the museum and its associated practices undergo constant transformation in response to broader societal and technical developments. This is especially true of museums holding ethnographic, world culture, or world art collections. One of the most significant developments in such museums in recent years has been the changing understanding of the relationships between ethnographic collections and the so‐called source or originating communities from whom they were obtained. Another key development has been the exploitation of digital technologies by museums, which has significantly widened access to these collections and facilitated more reciprocal forms of knowledge exchange around them. This chapter discusses a recent project that has sought to bring these two transformative spheres of museum practice together in order to explore the possibility of the museum's own transformative capacity as an agent of positive social change. The Reanimating Cultural Heritage project, which led to the creation of the digital heritage resource sierraleoneheritage.org, entailed a reconsideration of the value of dispersed and digitally remediated collections, and raised key questions concerning the adequacy of museological concepts such as the source community to fully enounce the relationships between differently situated stakeholders and these collections.
With and Without Walls: Photographic Reproduction and the Art Museum
Authors: Henning Michelle
Published: December 2013

This chapter explores ideas and arguments about the proliferation and circulation of photographic and digital reproductions of art. The existence of widely circulated reproductions before photography, and the early mingling of photography with painting and printmaking, demonstrates that the distinction between photographic reproduction and handmade original was always blurred. Even so, this distinction has informed thinking about art, reproduction, and museums. In the early twentieth century artists, curators, and art theorists thought that photography and photomechanical reproduction threatened the original work of art. The concepts of originality, aura, facture, and style became key, to distinguish photomechanical prints from valuable artworks, photography from painting. A new way of seeing seemed to be emerging – which treated reproduction as transparent, placed high value on originality and facture, and found new stylistic connections between images in the “museum without walls.” Modern forms of attention seemed to challenge the individualistic model of aesthetic contemplation enshrined in museums. Building on these early ideas, and situating them in relation to more recent discussion of digital and electronic media, this chapter argues that new practices of personal photography and image sharing require us to reimagine the museum with and without walls as potentially more “delirious,” playful, anarchic, and plural.
Open and Closed Systems: New Media Art in Museums and Galleries
Authors: Graham Beryl
Published: December 2013

Museums might be familiar with the idea of their audiences using new media technology as part of an officially authored marketing device, gallery guide, or a collections interface, but when the technology is used for art rather than interpretation, then boundaries can be pushed in interesting and productive ways. The particular behaviors of new media, including Steve Dietz's characteristics of connectivity, computability, and interactivity, fundamentally change the ways in which curators need to deal with time and space, such as the online and offline practices of San Francisco Museum of Modern Art, the Victoria and Albert Museum, Arnolfini, the Guggenheim Museum'sYouTube Playproject, and Yukiko Shikata'sMobLab. It is this rethinking of the role of the visitor, the artist, and the curator which is perhaps the most significant recent development associated with media in the museum, and new media in particular, leading to a consideration of whether museum systems are “open” or “closed.” Examples from artists and curators dealing with audience participation are discussed, including The People Speak, Robert Morris, Osman and Omar Khan, and Rafael Lozano‐Hemmer. Further examples, where audiences act as co‐creators, documenters, or curators of art, also challenge ideas of openness, includingThe Art of ParticipationandShredder. If openness exists beyond merely fashionable rhetoric, then there are critical delineations to be drawn between openness to artists or visitors, and also between differing levels of interaction, participation, or perhaps even control of decision‐making. This chapter offers understandings from new media, including Paul Baran's network models, and open source methods of production.
Keeping Objects Live
Authors: Candlin Fiona
Published: December 2013

It is often assumed that museum exhibits are inert but, in contrast to artifacts in most mainstream institutions, those at the Museum of Witchcraft, the Valiant Soldier community museum, and the Dartmoor Prison Museum are felt to be fully functioning and, to some extent, potent or dangerous. In order to consider why this is the case, this chapter investigates how museums are considered to “kill off” their exhibits and why this process does not occur in these small, independent organizations. Notably, the three venues have few or no paid members of staff and limited opportunities for gaining state funding. Operating largely independently of the public sector, they have no need to adopt official priorities and, in consequence, their modes of practice differ from those encountered in major institutions. They also have close links to their immediate location and communities. Focusing on these museums, therefore, raises the possibility that the “death” of objects is not a necessary condition but that their demise depends on the specific character and circumstances of display.
The Metallome
Authors: Gladyshev Vadim N. , Zhang Yan
Published: December 2013

Biological transition metals are essential for all organisms but are needed in very small quantities. They are thought to play important roles in a variety of biological processes. A lot of effort in this field has gone into experimental studies of metal utilization pathways and metalloproteins, whereas computational and systems biology analyses of the metallomes (or metalloproteomes) have been limited. In this article, the authors focus on the recent advances in bioinformatics, and especially comparative genomics, which provide significant insights into evolution and function of metal utilization. These studies suggest that zinc and iron are used by all or almost all living organisms, whereas most other metals, such as copper, molybdenum, nickel, and cobalt, show scattered occurrence in various groups of organisms. Different environmental and other factors have been suggested to affect the utilization of metals and evolution of metalloprotein families. We also discuss recent advances in high throughput ionomics studies in eukaryotes. Overall, bioinformatics analyses of metallomes provide a foundation for the systems level understanding of metal metabolism as well as for exploring the functions and evolutionary trends in metal utilization.
Nickel, Cobalt Transport in Prokaryotes
Authors: Eitinger Thomas
Published: December 2013

Nickel and cobalt ions are required by prokaryotes for incorporation into diverse enzymes involved in central metabolic reactions. The metal ions are taken up from the environment by selective high‐affinity primary and secondary active transport systems. These systems discriminate between Ni
2+
and Co
2+
on the one side and other divalent transition metal ions on the other side. Despite similar properties of Ni
2+
and Co
2+
, a couple of primary and secondary uptake systems exist that efficiently distinguish even between those two cations. Relevant transporters include canonical and energy‐coupling factor‐type ATP‐binding cassette importers and members of the nickel/cobalt transporter family and relatives of the latter. Recent advances include the discovery of active transport of nickel and cobalt across the outer membrane of gram‐negative bacteria by TonB‐dependent systems and the tentative identification of an unusual metallophore for nickel uptake. Export systems that avoid cellular overload with nickel and cobalt ions comprise multimeric systems of the resistance‐nodulation‐cell division family, secondary active systems from different subgroups, and P‐type ATPases.
Copper(I) ATPases: Transport Mechanism and Cellular Functions in Bacteria
Authors: Argüello José M. , Padilla‐Benavides Teresita , Collins Jessica M.
Published: December 2013

Transmembrane transporters are basic components of homeostatic systems that determine the cellular distribution of transition‐metal ions. Among these, P1B1‐type ATPases mediate cytoplasmic Cu(I) efflux. Prokaryotic Cu(I)‐ATPases are powerful tools to study the mechanism of transport and metal homeostasis in cells. They are similar to the eukaryotic homologs that play various unique roles in simple bacterial systems. Structural and functional characterizations of bacterial Cu(I)‐ATPases have shown the transport stoichiometry, metal coordination during transport, regulation by cytoplasmic metal‐sensing domains, and the mechanism of chaperone‐mediated Cu(I) access to transport sites. The high resolution structure ofLegionella pneumophilaCu(I)‐ATPase has revealed how the canonical structure of P‐type ATPases has adapted to enable Cu(I)‐transfer and translocation through a mechanism similar to the classical Albers–Post model. In addition, the importance of bacterial Cu(I)‐ATPases to the assembly of periplasmic and secreted metalloproteins is now clear, suggesting the participation of periplasmic Cu(I)‐trafficking proteins, which not only buffer the metal but also direct Cu(I) to the correct target apoproteins. Here, we discuss how the attributes of Cu(I)‐ATPases contribute to novel copper transport and homeostasis models.
Iron in Plants
Authors: Weng Jessica B. , Guerinot Mary Lou
Published: December 2013

Iron is essential for plant growth, and plant‐based foods are the primary source of iron in the human diet. Hence, understanding how plants take up iron, distribute iron, and regulate iron deficiency responses is important for both plant health and human nutrition. This chapter will review the two major strategies of iron uptake in plants as well as how iron is transported and compartmentalized to maintain iron homeostasis. We will also review recent approaches for reducing micronutrient deficiencies using biofortification.
Manganese in Neurodegeneration
Authors: Avila Daiana Silva , Puntel Robson Luiz , Soares Felix Antunes , da Rocha Joao Batista Teixeira , Aschner Michael
Published: December 2013

Manganese (Mn) is an important metal for mammalian organisms, necessary for development, brain function, and optimally functional antioxidant systems. Mn is required as cofactor for the proper functioning of multiple enzymes. However, excessive amounts of intracellular Mn preferentially accumulate in mitochondria, where it may cause oxidative stress and ensuing cellular dysfunction. In the brain, Mn also oxidizes dopamine, causing the death of dopaminergic neurons. Subjects exposed to high Mn levels from occupational or environmental sources may show symptoms resembling a parkinsonian‐like syndrome, referred to as manganism. This article reviews basic concepts on the essentiality and function of Mn as well as its mechanisms of neurodegeneration.
Iron Sequestration in Immunity
Authors: Correnti Colin , Strong Roland K.
Published: December 2013

Almost every living thing requires iron (including aerobic, and facultative and obligate anaerobic bacteria) because iron plays key roles in a broad range of physiologically essential molecular processes ranging from catalysis, oxygen transport, and oxidative phosphorylation to structuring proteins. The useful redox properties of iron enable its utility in these molecular functions, but its scarcity in the environment in bioavailable forms, coupled with the double‐edged nature of its redox properties (which can lead to the generation of cytotoxic reactive oxygen species), require complicated acquisition, transport, and storage mechanisms, both by environmental microbes and by higher animals. These iron acquisition mechanisms come into direct conflict when microbes compete with each other for this scarce resource in the environment and when pathogenic bacteria infect animal hosts. A very simple and potently effective strategy employed by many animals to ward off bacterial and fungal infections, at least in the early stages while pathogen‐specific adaptive immune responses are developing, is to further constrict the already tightly controlled endogenous iron budget, starving invading microbial pathogens of needed iron. The complicated system of endogenous iron transport permits these changes in response to infection. However, in a countering response, successful pathogens have developed a variety of strategies to evade this defense, the simplest and most dramatic of which is perhaps exemplified by the spirochete
Borrelia burgdorferi
, the causative agent of Lyme disease, which has evolved away from the need for any iron whatsoever. Bacteria and protists have also developed sophisticated and complicated mechanisms to access heme‐associated iron directly. However, other strategies developed by microbes to compete with each other for iron in the environment, mediated by small‐molecule iron chelators called siderophores, are, in turn, useful to access the otherwise restricted pool of iron in transit in the body, carried as complexes with specific iron‐binding proteins. To counter siderophore‐mediated iron pilfering, animals have developed a panel of siderophore‐specific binding proteins (siderocalins) to steal the iron (and siderophore) back. To subvert this counterstrategy, successful pathogenic bacteria modify their siderophores into stealthy forms that cannot bind to host siderocalins. As with any arms race (e.g., the process of evolution), the cycle of response/counterresponse is likely to continue ad infinitum.
Molybdenum: Nitrogenase Cofactor Assembly
Authors: Wiig Jared A. , Lee Chi Chung , Ribbe Markus W. , Hu Yilin
Published: December 2013

The assembly of the nitrogenase iron–molybdenum (FeMo) cofactor (M‐cluster) has been described as one of the most complex processes in bioinorganic chemistry. While initial genetic approaches identified the key players in M‐cluster assembly, biochemical and structural investigations in the past few years further elucidated the inner workings of this process. Specifically, the role of the radical
S
‐adenosyl‐methionine (SAM) enzyme NifB, as well as the crystal structure of the scaffold protein NifEN, has been defined recently, leading to a better understanding of the key steps of M‐cluster assembly that occur on these two assembly proteins. Here, the authors highlight the recent advances in their understanding of M‐cluster assembly, with a focus on the roles of NifB and NifEN in this process.
Copper in Mitochondria
Authors: Vest Katherine E. , Cobine Paul A.
Published: December 2013

Copper is an essential micronutrient required in mitochondria for energy production in aerobic eukaryotes. Copper is a cofactor in the enzymes cytochrome
c
oxidase and superoxide dismutase. Metallochaperone proteins such as Cox17, Sco1, Cox11, and Ccs1 transiently bind copper in the intermembrane space to deliver it to cytochrome
c
oxidase or superoxide dismutase. However, the bulk of copper is in a labile pool within the mitochondrial matrix. This pool of copper is required for activation of intermembrane space cuproenzymes. Matrix copper is bound to an anionic, fluorescent molecule known as the copper ligand. Using basic framework discovered in
Saccharomyces cerevisiae
as a context, the current state of knowledge for each of these pools of copper will be presented. In addition, the authors highlight the unexpected activities of the
SCO
proteins in cellular copper homeostasis in mammals and speculate on the unresolved questions of mitochondrial copper.
Magnetic Targeting Enhanced Theranostic Strategy Based on Multimodal Imaging for Selective Ablation of Cancer
Authors: Li Zhiwei , Yin Shengnan , Cheng Liang , Yang Kai , Li Yonggang , Liu Zhuang
Published: December 2013

The booming development of nanomedicine offers great opportunities for cancer diagnostics and therapeutics. Herein, a magnetic targeting‐enhanced cancer theranostic strategy using a multifunctional magnetic‐plasmonic nano‐agent is developed, and a highly effective in vivo tumor photothermal therapy, which is carefully planed based on magnetic resonance (MR)/photoacoustic (PA) multimodal imaging, is realized. By applying an external magnetic field (MF) focused on the targeted tumor, a magnetic targeting mediated enhanced permeability and retention (MT‐EPR) effect is observed. While MR scanning provides tumor localization and reveals time‐dependent tumor homing of nanoparticles for therapeutic planning, photoacoustic imaging with higher spatial resolution allows noninvasive fine tumor margin delineation and vivid visualization of three dimensional distributions of theranostic nanoparticles inside the tumor. Utilizing the near‐infrared (NIR) plasmonic absorbance of those nanoparticles, selective photothermal tumor ablation, whose efficacy is predicted by real‐time infrared thermal imaging intra‐therapeutically, is carried out and then monitored by MR imaging for post‐treatment prognosis. Overall, this study illustrates the concept of imaging‐guided MF‐targeted photothermal therapy based on a multifunctional nano‐agent, aiming at optimizing therapeutic planning to achieve the most efficient cancer therapy.
Kinetic modeling of hemicellulose hydrolysis in the presence of homogeneous and heterogeneous catalysts
Authors: Salmi Tapio , Murzin Dmitry Yu. , Mäki‐Arvela Päivi , Kusema Bright , Holmbom Bjarne , Willför Stefan , Wärnå Johan
Published: December 2013

Kinetic models were developed for the hydrolysis of O‐acetyl‐galactoglucomannan (GGM), a hemicellulose appearing in coniferous trees. Homogeneous and heterogeneous acid catalysts hydrolyze GGM at about 90°C to the monomeric sugars galactose, glucose, and mannose. In the presence of homogeneous catalysts, such as HCl, H2SO4, oxalic acid, and trifluoroacetic acid, the hydrolysis process shows a regular kinetic behavior, while a prominent autocatalytic effect was observed in the presence of heterogeneous cation‐exchange catalysts, Amberlyst 15 and Smopex 101. The kinetic models proposed were based on the reactivities of the nonhydrolyzed sugar units and the increase of the rate constant (for heterogeneous catalysts) as the reaction progresses and the degree of polymerization decreases. General kinetic models were derived and special cases of them were considered in detail, by deriving analytical solutions for product distributions. The kinetic parameters, describing the autocatalytic effect were determined by nonlinear regression analysis. The kinetic model described very well the overall kinetics, as well as the product distribution in the hydrolysis of water soluble GGM by homogeneous and heterogeneous catalysts. The modelling principles developed in the work can be in principle applied to hydrolysis of similar hemicelluloses as well as starch and cellulose. © 2014 American Institute of Chemical Engineers AIChE J, 60: 1066–1077, 2014
Longitudinal variation of circulating irisin after an energy restriction‐induced weight loss and following weight regain in obese men and women
Authors: Crujeiras Ana B. , Pardo María , Arturo Roca‐Rivada , Santiago Navas‐Carretero , Zulet M. Angeles , Martínez J. Alfredo , Casanueva Felipe F
Published: December 2013

Objective

The recently discovered peptide irisin has been hypothesized to be a regulator of body metabolism. The objective of this work was to evaluate whether circulating human irisin levels are modulated by body size and changes in adiposity during an energy restriction treatment and the subsequent weight regain.

Methods

A group of 94 obese patients (50 men, 44 women; 49.4 ± 9.4 years; BMI 35.6 ± 4.5 kg/m2) participated in a weight loss program following an 8‐week hypocaloric diet (−30% energy expenditure) with a weight maintenance follow‐up. The patients were evaluated at 0, 8, and 24 weeks after starting treatment. In addition, 48 normal‐weight subjects (16 men, 32 women; 35.71 ± 8.8 years; BMI 22.9 ± 2.2 kg/m2) participated as controls. Plasma irisin, body weight, body composition, and hormones controlling energy homeostasis were measured.

Results

Irisin levels were higher in obese subjects (353.1 ± 18.6 ng/mL) than in those of normal‐weight (198.4 ± 7.8 ng/mL;P ≤ 0.001) and were also higher in men (340.9 ± 20 ng/mL) than in women (267.6 ± 12 ng/mL;P < 0.05). Moreover, irisin plasma levels were significantly correlated with high levels of direct and indirect adiposity markers, such as weight, BMI, waist circumference, and fat mass, as measured by bioimpedance, but not with height or leptin levels. Interestingly, irisin levels paralleled body weight reduction after the dietary treatment (week 8) and again returned to the baseline levels at 24 weeks in those patients regaining the lost weight.

Conclusions

Irisin strongly reflects body fat mass, suggesting that the irisin circulating levels are conditioned by adiposity level. Am. J. Hum. Biol. 26:198–207, 2014. © 2013 Wiley Periodicals, Inc.
Pituitary deficiency and congenital infiltrating lipomatosis of the face in a girl with deletion of chromosome 1q24.3q31.1
Authors: Capra V. , Severino M. , Rossi A. , Nozza P. , Doneda C. , Perri K. , Pavanello M. , Fiorio P. , Gimelli G. , Tassano E. , Di Battista E.
Published: December 2013

Interstitial deletions of the long arm of chromosome 1 are rare and they are classified as proximal or intermediate. The intermediate interstitial deletions span 1q24–1q32. We describe a 6‐year‐old girl with multiple pituitary hormone deficiency, severe cognitive impairment, bilateral cleft lip and palate, midline facial capillary malformation, erythema of hands and feet and dysplastic cranial vessels, low anti‐thrombin III activity, hemifacial overgrowth due to progressive infiltrating lipomatosis with bone overgrowth, marked vascular proliferation and erythema of hands and feet, and abnormal cranial vessels. The girl's karyotype showed an apparently de novo interstitial deletion 1q24.3q31.1, which was defined by array‐CGH. The deleted region contains numerous genes, but only eight (CENPL, LHX4, LAMC1, LAMC2, PTGS2, ANGPTL1, TNN, and TNR) are good candidates to explain, at least partially, the phenotype of the proposita. We, therefore, discuss the involvement of these genes and the observed phenotype. © 2013 Wiley Periodicals, Inc.
Inherited dup(17)(p11.2p11.2): Expanding the phenotype of the Potocki–Lupski syndrome
Authors: Magoulas Pilar L. , Liu Pengfei , Gelowani Violet , Soler‐Alfonso Claudia , Kivuva Emma C. , Lupski James R. , Potocki Lorraine
Published: December 2013

Potocki–Lupski syndrome (PTLS, OMIM: 610883) is a microduplication syndrome characterized by infantile hypotonia, failure to thrive, cardiovascular malformations, developmental delay, intellectual disability, and behavior abnormalities, the latter of which can include autism spectrum disorder. The majority of individuals with PTLS harbor a de novo microduplication of chromosome 17p11.2 reciprocal to the common recurrent 3.6 Mb microdeletion in the Smith–Magenis syndrome critical region. Here, we report on the transmission of the PTLS duplication across two generations in two separate families. Individuals in these families presented initially with developmental delay, behavior problems, and intellectual disability. We provide a detailed review of the clinical and developmental phenotype of inherited PTLS in both families. This represents the second report (second and third families) of PTLS in a parent–child pair and exemplifies the under‐diagnosis of this and likely other genetic conditions in adults with intellectual disability and/or psychiatric disorders. © 2013 Wiley Periodicals, Inc.
Late diagnosis of lateral meningocele syndrome in a 55‐year‐old woman with symptoms of joint instability and chronic musculoskeletal pain
Authors: Castori Marco , Morlino Silvia , Ritelli Marco , Brancati Francesco , De Bernardo Carmelilia , Colombi Marina , Grammatico Paola
Published: December 2013

Lateral meningocele syndrome (LMS) is a rare hereditary connective tissue disorder characterized by pan‐spinal meningoceles, specific facial dysmorphism, skeletal and soft tissue abnormalities, and hypotonia and/or muscle weakness. LMS has been observed in eleven patients with two instances of vertical transmission, and seven sporadic cases with an age at diagnosis ranging from 25 months to 33 years. We report on a further observation of LMS in a 55‐year‐old woman presenting with a long history of joint instability, chronic musculoskeletal pain, and iatrogenic bladder and anorectal dysfunction due to irreversible nerve damage after surgical excision of a meningeal cyst. Her clinical characteristics are compared with those of previously reported patients, as well as two further cases originally diagnosed with Hajdu–Cheney and Ehlers–Danlos syndromes, but displaying typical features of LMS. © 2013 Wiley Periodicals, Inc.
Waardenburg syndrome type 4: Report of two new cases caused by SOX10 mutations in Spain
Authors: Fernández Raquel M. , Núñez‐Ramos Raquel , Enguix‐Riego Mª Valle , Román‐Rodríguez Francisco José , Galán‐Gómez Enrique , Blesa‐Sánchez Emilio , Antiñolo Guillermo , Núñez‐Núñez Ramón , Borrego Salud
Published: December 2013

Shah‐Waardenburg syndrome or Waardenburg syndrome type 4 (WS4) is a neurocristopathy characterized by the association of deafness, depigmentation and Hirschsprung disease. Three disease‐causing genes have been identified so far for WS4: EDNRB, EDN3, and SOX10. SOX10 mutations, found in 45–55% of WS4 patients, are inherited in autosomal dominant way. In addition, mutations in SOX10 are also responsible for an extended syndrome involving peripheral and central neurological phenotypes, referred to as PCWH (peripheral demyelinating neuropathy, central dysmyelinating leucodystrophy, Waardenburg syndrome, Hirschsprung disease). Such mutations are mostly private, and a high intra‐ and inter‐familial variability exists. In this report, we present a patient with WS4 and a second with PCWH due to SOX10 mutations supporting again the genetic and phenotypic heterogeneity of these syndromes. Interestingly, the WS4 family carries an insertion of 19 nucleotides in exon 5 of SOX10, which results in distinct phenotypes along three different generations: hypopigmentation in the maternal grandmother, hearing loss in the mother, and WS4 in the proband. Since mosaicism cannot explain the three different related‐WS features observed in this family, we propose as the most plausible explanation the existence of additional molecular events, acting in an additive or multiplicative fashion, in genes or regulatory regions unidentified so far. On the other hand, the PCWH case was due to a de novo deletion in exon 5 of the gene. Efforts should be devoted to unravel the mechanisms underlying the intrafamilial phenotypic variability observed in the families affected, and to identify new genes responsible for the still unsolved WS4 cases. © 2013 Wiley Periodicals, Inc.
Combined immunodeficiency in a 3‐year‐old boy with 16p11.2 and 20p12.2‐11.2 chromosomal duplications
Authors: Batanian Jacqueline R. , Braddock Stephen R. , Christensen Katherine , Knutsen Alan P.
Published: December 2013

We report for the first time on a 3‐year‐old boy with paternally inherited 212.85 kb—16p11.2 and 7.8 Mb—20p12.2‐11.23 interstitial microduplications associated with having congenital cardiac defect, dysmorphic facial features, and combined T‐, B‐, and NK cell immunodeficiency. In addition the 7.8 Mb—20p12.2‐11.23 microduplication is unique showing novel breakpoints among all partial trisomy/duplication 20p reported to date, narrowing down the critical region for trisomy 20p syndrome. © 2013 Wiley Periodicals, Inc.
Leaf chemistry as a predictor of primate biomass and the mediating role of food selection: A case study in a folivorous lemur (Propithecus verreauxi)
Authors: Simmen Bruno , Tarnaud Laurent , Marez André , Hladik Annette
Published: December 2013

Folivorous primate biomass has been shown to positively correlate with the average protein‐to‐fiber ratio in mature leaves of tropical forests. However, studies have failed to explain the mismatch between dietary selection and the role of the protein‐to‐fiber ratio on primate biomass; why do not folivores always favor mature leaves or leaves with the highest protein‐to‐fiber ratio? We examined the effect of leaf chemical characteristics and plant abundance (using transect censuses; 0.37 ha, 233 trees) on food choices and nutrient/toxin consumption in a folivorous lemur (Propithecus verreauxi) in a gallery forest in southern Madagascar. To assess the nutritional quality of the habitat, we calculated an abundance‐weighted chemical index for each chemical variable. Food intake was quantified using a continuous count of mouthfuls during individual full‐day follows across three seasons. We found a significant positive correlation between food ranking in the diet and plant abundance. The protein‐to‐fiber ratio and most other chemical variables tested had no statistical effect on dietary selection. Numerous chemical characteristics of the sifaka's diet were essentially by‐products of generalist feeding and “low energy input/low energy crop” strategy. The examination of feeding behavior and plant chemistry in Old World colobines and folivorous prosimians in Madagascar suggests that relative lack of feeding selectivity and high primate biomass occur when the average protein‐to‐fiber ratio of mature leaves in the habitat exceeds a threshold at 0.4. Am. J. Primatol. 76:563–575, 2014. © 2013 Wiley Periodicals, Inc.
Allergy education in otolaryngology residency: a survey of program directors and residents
Authors: Bailey Sarah E. , Franzese Christine , Lin Sandra Y.
Published: December 2013

Background

The purpose of this study was to survey program directors of the accredited otolaryngology residency programs and resident attendees of the 2013 American Academy of Otolaryngic Allergy (AAOA) Basic/MOC Course regarding resident education and participation as well as assessment of competency in otolaryngic allergy and immunotherapy.

Methods

A multiple‐choice questionnaire was sent to all accredited otolaryngology residency training programs in the United States as part of resident attendance at the 2013 AAOA CORE Basic/MOC Course. Following this, a similar multiple‐choice survey was sent to all resident attendees from the programs that responded positively.

Results

Program directors reported that 73% of their academic institutions offer allergy testing and immunotherapy. More PDs than residents indicated that residents participate in allergy practice and perform/interpret skin testing and in vitro testing, and more residents (85%) than program directors (63%) reported inadequate or no allergy training. Program directors and residents equally indicated that residents do not calculate immunotherapy vial formulations or administer immunotherapy injections. The majority of program directors indicated that resident competency in allergy was assessed through direct observation, whereas residents more commonly perceived that no assessment of competency was being performed for any portion of allergy practice.

Conclusion

This survey demonstrates a discrepancy between program directors and residents regarding resident involvement and adequacy of training in the allergy practice. Although the majority of otolaryngology residencies report offering otolaryngic allergy services and education, the vast majority of residents report inadequate allergy training and less participation in an allergy practice compared to the majority of program directors.
Synthesis and characterization of surface‐grafted poly(N‐isopropylacrylamide) and poly(carboxylic acid)—Iron particles via atom transfer radical polymerization for biomedical applications
Authors: Sutrisno Joko , Fuchs Alan , Evrensel Cahit
Published: December 2013

This research relates to the preparation and characterization of surface grafted poly(N‐isopropylacrylamide) and poly(carboxylic acid)–micron‐size iron particles via atom transfer radical polymerization (ATRP). The surface grafted polymers–iron particles result in multifunctional materials which can be used in biomedical applications. The functionalities consist of cell targeting, imaging, drug delivery, and immunological response. The multifunctional materials are synthesized in two steps. First, surface grafting is used to place polymer molecules on the iron particles surface. The second step is conjugation of the bio‐molecules onto the polymer backbone. Fourier transform infrared (FTIR) spectroscopy and scanning electron microscopy were used to confirm the presence of polymers on the iron particles. The thickness of the grafted polymers and glass transition temperature of the surface grafted polymers were determined by transmission electron microscopy (TEM) and differential scanning calorimetry (DSC). The covalent bond between grafted polymers and iron particles caused higher glass transition temperature as compared with nongrafted polymers. The ability to target the bio‐molecule and provide fluorescent imaging was simulated by conjugation of rat immunoglobulin and fluorescein isothiocyanate (FITC) labeled anti‐rat. The fluorescence intensity was determined using flow cytometry and conjugated IgG‐FITC anti‐rat on iron particles which was imaged using a fluorescence microscopy. © 2013 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2014, 131, 40176.
Salt‐free softening by thermo‐reversible ion‐adsorbing hydrogels
Authors: Custers Johannes P. A. , Stemkens Léon F. S. , Sablong Rafael , van Asseldonk Dirk T. A. , Keurentjes Jos T. F.
Published: December 2013

In this article, we demonstrate the properties of a hydrogel capable of adsorbing calcium cations from aqueous streams at ambient temperatures (10–25°C) and showing almost complete desorption of the bound calcium ions at slightly elevated temperatures (40–50°C). Successful breakthrough experiments in a fixed bed column set‐up show the potential of the hydrogel in softening water streams from 150 ppm toward <10 ppm hardness levels. The regeneration ability is based on the thermo‐reversible internal ion pair–formation mechanism of the hydrogel. Additionally, it was shown that the thermo‐reversible adsorption mechanism was successful for magnesium and copper ions too. The exploration of the properties of hydrogel CS‐1B14 is the first step in the development of sustainable water‐softening processes, both at industrial scale as well as for household applications, using low‐grade residual heat to regenerate these gels. In this way, the use of concentrated brine streams, acid/caustic treatment, or high regeneration temperatures is avoided. © 2013 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2014, 131, 40216.
Stable Encapsulation of Acrylate Esters in Networked Molecular Capsules
Authors: Ning Guo‐Hong , Inokuma Yasuhide , Fujita Makoto
Published: December 2013

Reactive acrylate esters were encapsulated in the cavity of networked molecular capsules in a single‐crystal‐to‐single‐crystal fashion. Owing to the encapsulation effect, acrylates inside the capsules do not undergo polymerization upon irradiation with UV light or heating, while the guest molecules can be quantitatively extracted by treatment with toluene.
The 2.05‐helix in hetero‐oligopeptides entirely composed of Cα,α‐disubstituted glycines with both side chains longer than methyls
Authors: Crisma Marco , Peggion Cristina , Moretto Alessandro , Banerjee Raja , Supakar Subhrangshu , Formaggio Fernando , Toniolo Claudio
Published: December 2013

The existence of the very uncommon, but potentially quite interesting, multiple, consecutive fully‐extended conformation (2.05‐helix) has been already clearly demonstrated in homo‐oligopeptides based on quaternary α‐amino acids with both side chains longer than methyls, but not cyclized on the α‐carbon atom. To extend the scope of this research, in this work we investigated the occurrence of this flat 3D‐structure in hetero‐oligopeptides, each composed of two or three different residues of that class. The synthesis of a terminally protected peptide series to the tetrapeptide level was carried out by solution methods. The resulting oligomers were chemically and conformationally characterized. The data obtained point to an overwhelming population of the fully‐extended conformation in CDCl3. However, a solvent‐driven switch to a predominant 310‐helical structure was seen in CD3CN. A delicate, local balance between these two conformations is confirmed to occur in the crystalline state. Molecular dynamics simulations in CHCl3 on a hetero‐tetrapeptide converged to the fully‐extended conformation even starting from the 310‐helical structure. © 2013 Wiley Periodicals, Inc. Biopolymers (Pept Sci) 102: 145–158, 2014.
MRI-defined height of rectal tumours
Authors: Keller D S , Paspulati R , Kjellmo A , Rokseth K M , Bankwitz B , Wibe A , Delaney C P
Published: December 2013

Background

There is no standard for reporting rectal cancer distances from the distal resection margin in the literature. The objective was to demonstrate the importance of rectal cancer measurement from a standardized point.

Methods

Review of databases at two international institutions identified 50 patients with rectal adenocarcinoma within 15 cm of the anal verge (AV), who had preoperative magnetic resonance imaging (MRI) and underwent surgery with curative intent. Expert radiologists reviewed the magnetic resonance images for anatomical distances from the anorectal ring (ARR) to the AV, from the ARR to the dentate line (DL), and from the DL to the AV. Anatomical measurements were compared with preoperative measurements to assess reporting inconsistencies.

Results

Fifty patients with rectal adenocarcinoma were included in the study. The mean(s.d.) anatomical distance was 1·66(0·61) cm from the ARR to the DL, 3·78(0·61) cm from the ARR to the AV (maximum 5·5 cm) and 2·11(0·10) cm from the DL to the AV. The mean radiological distance from the distal tumour was 2·90(1·60) (median 3·2, range 0–7·5) cm to the ARR, 4·36(3·20) (median 4·2, range −0·5 to 12·8) cm to the DL and 6·13(3·39) (median 6·0, range 0–14·1) cm to the AV. There was a significant difference in the distal tumour margin between measurements made by the expert radiologists and reported preoperative measurements (P < 0·001). Significant differences were also found between the expert radiologists' MRI and rigid proctoscopic measurements (P = 0·025).

Conclusion

There was up to 5·5 cm variation, depending on which landmark was chosen for reporting the distal margin of rectal cancer. This has potential implications for surgical planning, interpreting radiological images and comparative studies.
Boards and Sustainability: the Contingent Influence of Director Interlocks on Corporate Environmental Performance
Authors: Ortiz‐de‐Mandojana Natalia , Aragon‐Correa Juan Alberto
Published: December 2013

This paper highlights the importance of a firm's board with respect to sustainability issues by analysing the relationship between director interlocks, i.e. directors who simultaneously belong to the boards of directors of several companies, and a firm's environmental performance. The previous literature has focused on the influence of firm‐level resources on corporate environmental performance. This study utilizes insights from a resource‐based view and research on social capital to demonstrate that the environmental performance of a firm is also influenced by the difficult‐to‐imitate capabilities that are embedded in the network relationships of its directors. Our results support a contingency perspective of the social capital theory that finds that director interlocks are positively connected with the environmental performance of a firm in two specific situations: (1) when the firm is linked to a larger parent company and (2) in cases of low and high levels of interlock diversity. Copyright © 2013 John Wiley & Sons, Ltd and ERP Environment.
In Situ Observation of Cu–Ni Alloy Nanoparticle Formation by X‐Ray Diffraction, X‐Ray Absorption Spectroscopy, and Transmission Electron Microscopy: Influence of Cu/Ni Ratio
Authors: Wu Qiongxiao , Duchstein Linus D. L. , Chiarello Gian Luca , Christensen Jakob M. , Damsgaard Christian D. , Elkjær Christian F. , Wagner Jakob B. , Temel Burcin , Grunwaldt Jan‐Dierk , Jensen Anker D.
Published: December 2013

Silica‐supported, bimetallic Cu–Ni nanomaterials were prepared with different ratios of Cu to Ni by incipient wetness impregnation without a specific calcination step before reduction. Different in situ characterization techniques, in particular transmission electron microscopy (TEM), X‐ray diffraction (XRD), and X‐ray absorption spectroscopy (XAS), were applied to follow the reduction and alloying process of Cu–Ni nanoparticles on silica. In situ reduction of Cu–Ni samples with structural characterization by combined synchrotron XRD and XAS reveals a strong interaction between Cu and Ni species, which results in improved reducibility of the Ni species compared with monometallic Ni. At high Ni concentrations silica‐supported Cu–Ni alloys form a homogeneous solid solution of Cu and Ni, whereas at lower Ni contents Cu and Ni are partly segregated and form metallic Cu and Cu–Ni alloy phases. Under the same reduction conditions, the particle sizes of reduced Cu–Ni alloys decrease with increasing Ni content. Estimates of the metal surface area from sulfur chemisorption and from the XRD particle size generally agree well on the trend across the composition range, but show some disparity in terms of the absolute magnitude of the metal area. This work provides practical synthesis guidelines towards preparation of Cu–Ni alloy nanomaterials with different Cu/Ni ratios, and insight into the application of different in situ techniques for characterization of the alloy formation.