bluuebunny/crossref_metadata_2025_split huggingface dataset:
What, or Where, Is the (Museum) Object?: Colonial Encounters in Displayed Worlds of Things
Authors: Dudley Sandra H. 
Published: December 2013

AbstractThis 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 Metallome
Authors: Gladyshev Vadim N. , Zhang Yan 
Published: December 2013

AbstractBiological 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

Abstract

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

AbstractTransmembrane 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

Abstract
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

AbstractManganese (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

Abstract

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

Abstract

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

Abstract

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.
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

ObjectiveThe 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.MethodsA 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.ResultsIrisin 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.ConclusionsIrisin 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

ABSTRACTInterstitial 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

ABSTRACTPotocki–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

ABSTRACTLateral 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

AbstractShah‐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

ABSTRACTWe 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.
Allergy education in otolaryngology residency: a survey of program directors and residents
Authors: Bailey Sarah E. , Franzese Christine , Lin Sandra Y. 
Published: December 2013

BackgroundThe 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.MethodsA 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.ResultsProgram 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.ConclusionThis 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.