bluuebunny/crossref_metadata_2025 huggingface dataset:
Guttation: path, principles and functions
Authors: Singh Sanjay
Published: February 2014

Guttation is a process of natural secretion of fluid from leaves via specialised structures called ‘hydathodes’, which are located at the tips, margins, and adaxial and abaxial surfaces of leaves. Hydathodes form natural openings but, unlike stomata, are open permanently and offer little resistance to the flow of fluid out of leaves. Each hydathode is formed of colourless cells, and appears as stomata-like pores in the epidermis or epithem, also known as ‘transfer tissue’. The cells of epithem are soft and made of loosely arranged thin-walled parenchyma cells and without chloroplast, and are involved in absorption and secretion. Internally, they are connected by tracheary endings to a large chamber with masses of thin-walled parenchymatous tissue surrounded by a sheath layer. Ultrastructurally, the epithem cells have a dense cytoplasm, numerous mitochondria, an extensive endoplasmic reticulum system, many small Golgi-derived vesicles, numerous peroxisomes, and are interconnected by abundant plasmodesmata. Functionally, there are two types of hydathodes, namely, epidermal ones that actively exude fluid, and epithemal hydathodes that passively exude fluid. Natural guttation is often observed during early morning or late hours of the day. However, it can also be induced as desired in intact or excised plants under pneumatic pressure. Earlier notions regarding harmful effects on plants of guttation have now been addressed by botanical and physiological research discoveries regarding the basic and practical utility of guttation. This knowledge could lead to new health care applications on the one hand and ease global food-security concerns on the other.
PrometheusWiki Gold Leaf Protocol: gas exchange using LI-COR 6400
Authors: Evans John R. , Santiago Louis S.
Published: February 2014

This leaf gas exchange protocol enables light or CO2 response curves using a LI-COR LI-6400 portable photosynthesis system. The protocol originates in PrometheusWiki (http://prometheuswiki.publish.csiro.au/) where it has been tested and verified, and has received favourable user reviews. This reformatted and non-editable version is published as a Gold Leaf Protocol. For the most current version, including any user-commentary updates, readers may view the live version of the protocol at http://bit.ly/PWLicorGold.
EZH2 expands breast stem cells through activation of NOTCH1 signaling
Authors: Gonzalez Maria E. , Moore Heather M. , Li Xin , Toy Kathy A. , Huang Wei , Sabel Michael S. , Kidwell Kelley M. , Kleer Celina G.
Published: February 2014

Significance

Triple-negative breast cancers comprise 10% of invasive breast carcinomas but are responsible for a disproportionate number of deaths and remain poorly understood. Unfortunately, current therapies are only weakly effective, and the median disease-free survival is 4 y among young women. Clinical studies support the relevance of Enhancer of Zeste Homolog 2 (EZH2) overexpression to the progression of triple-negative breast carcinomas. Our study shows that EZH2 acts as an activator of the
NOTCH1
promoter and signaling to expand the stem cell pool, leading to accelerated breast cancer initiation and growth. We discovered that this function is independent of EZH2 histone methyltransferase activity and of its Polycomb Repressive Complex 2-binding partners, paving the way for novel therapeutic strategies.
Fourth class of convex equilateral polyhedron with polyhedral symmetry related to fullerenes and viruses
Authors: Schein Stan , Gayed James Maurice
Published: February 2014

Significance

The Greeks described two classes of convex equilateral polyhedron with polyhedral symmetry, the Platonic (including the tetrahedron, octahedron, and icosahedron) and the Archimedean (including the truncated icosahedron with its soccer-ball shape). Johannes Kepler discovered a third class, the rhombic polyhedra. Some carbon fullerenes, inorganic cages, icosahedral viruses, protein complexes, and geodesic structures resemble these polyhedra. Here we add a fourth class, “Goldberg polyhedra.” Their small (corner) faces are regular 3gons, 4gons, or 5gons, whereas their planar 6gonal faces are equilateral but not equiangular. Unlike faceted viruses and related carbon fullerenes, the icosahedral Goldberg polyhedra are nearly spherical. The reasoning and techniques presented here will enable discovery of still more classes of convex equilateral polyhedron with polyhedral symmetry.
Integrin α9 on lymphatic endothelial cells regulates lymphocyte egress
Authors: Ito Koyu , Morimoto Junko , Kihara Akio , Matsui Yutaka , Kurotaki Daisuke , Kanayama Masashi , Simmons Szandor , Ishii Masaru , Sheppard Dean , Takaoka Akinori , Uede Toshimitsu
Published: February 2014

Significance

The lymphatic system plays critical roles in immune surveillance by providing a route for circulating immune cells. α9 integrin is a signature molecule of lymphatic endothelial cells (LECs), and has been suggested to have a significant role in lymphatic functions. Here we found that in draining lymph nodes (LNs) of mice treated with complete Freund adjuvant, blockade of α9 integrin induced deficiency of lymphocytes in medullary and cortical sinuses, which is the characteristic feature of impaired lymphocyte egress from LNs. In addition, we found that stimulation of embryo-derived LECs with the α9 ligand tenascin-C induced secretion of sphingosine 1-phosphate, which is the critical factor for lymphocyte egress. Thus, we suggest a possible role of α9 integrin-dependent lymphocyte egress from inflamed LNs.
HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response
Authors: Rotblat Barak , Southwell Amber L. , Ehrnhoefer Dagmar E. , Skotte Niels H. , Metzler Martina , Franciosi Sonia , Leprivier Gabriel , Somasekharan Syam Prakash , Barokas Adi , Deng Yu , Tang Tiffany , Mathers Joan , Cetinbas Naniye , Daugaard Mads , Kwok Brian , Li Liheng , Carnie Christopher J. , Fink Dieter , Nitsch Roberto , Galpin Jason D. , Ahern Christopher A. , Melino Gerry , Penninger Josef M. , Hayden Michael R. , Sorensen Poul H.
Published: February 2014

Significance

Oxidative stress is an important contributor to aging-associated diseases including cancer and neurodegeneration, and antioxidant stress responses are critical to limit manifestations of these diseases. We report that the tumor suppressor Homologous to the E6-AP Carboxyl Terminus domain and Ankyrin repeat containing E3 ubiquitin–protein ligase 1 (HACE1) promotes activity of the transcription factor, nuclear factor erythroid 2-related factor 2 (NRF2), a master regulator of the antioxidative stress response. In Huntington disease patients, HACE1 is lost in the brain region most affected by the disease, namely the striatum, and restoring HACE1 functions in striatal cells expressing mutant Huntingtin protein provides protection against oxidative stress. Therefore, the tumor suppressor HACE1 is a new regulator of NRF2 and an emerging player in neurodegeneration.
B-
myb
is an essential regulator of hematopoietic stem cell and myeloid progenitor cell development
Authors: Baker Stacey J. , Ma’ayan Avi , Lieu Yen K. , John Premila , Reddy M. V. Ramana , Chen Edward Y. , Duan Qiaonan , Snoeck Hans-Willem , Reddy E. Premkumar
Published: February 2014

Significance

Hematopoiesis is a tightly regulated process by which hematopoietic stem cells (HSCs) give rise to mature cells. The balance between the propensity of HSCs to remain quiescent, to divide and generate more HSCs (self-renewal), or to divide and give rise to mature cells (differentiation) is essential for the long-term maintenance of blood cell formation. Mechanisms underlying cell fate decisions of HSCs are not completely understood. We observed that deletion of the B-
myb
gene leads to depletion of the HSC pool and losses of mature cells. Our further studies strongly suggest that these effects are due to defects in HSC proliferation and differentiation. We therefore identified B-
myb
as a critical component of the mechanism that balances self-renewal and differentiation of HSCs.
Bedrock composition regulates mountain ecosystems and landscape evolution
Authors: Hahm W. Jesse , Riebe Clifford S. , Lukens Claire E. , Araki Sayaka
Published: February 2014

Significance

This investigation focused on the factors that influence forest cover in the Sierra Nevada, California, where Giant Sequoia, the largest trees on Earth, grow in groves immediately next to expanses of rock devoid of soil and vegetation. The differences in forest cover correspond to twofold differences in erosion rates, suggesting that vegetation is an important regulator of landscape evolution across the region. Analyses presented here show that differences in forest cover can be explained by variations in geochemical composition of underlying bedrock. These results are important because they demonstrate that bedrock geochemistry is on par with climate as a regulator of vegetation in the Sierra Nevada and likely in other granitic mountain ranges around the world.
Day–night cycles and the sleep-promoting factor, Sleepless, affect stem cell activity in the
Drosophila
testis
Authors: Tulina Natalia M. , Chen Wen-Feng , Chen Jung Hsuan , Sowcik Mallory , Sehgal Amita
Published: February 2014

Significance

Daily rhythms in light coordinate many biological functions over the 24-h day, facilitating the adaptation of organisms to the environment. A crucial task accomplished routinely by all multicellular organisms is the maintenance of tissue homeostasis through the activity of adult stem cells, which generate new cells when required. Using a classic
Drosophila
stem cell niche, we show that the frequency of stem cell divisions is rhythmic in a day–night cycle. In addition, our data, which show that stem cell divisions are increased in a short-sleeping mutant, suggest that mechanisms regulating sleep–wake rhythms may also influence stem cell activity. Our findings provide links between environmental cycles, behavioral states, and maintenance of tissue integrity by stem cells.
Humanized-BLT mouse model of Kaposi’s sarcoma-associated herpesvirus infection
Authors: Wang Lin-Xu , Kang Guobin , Kumar Pankaj , Lu Wuxun , Li Yue , Zhou You , Li Qingsheng , Wood Charles
Published: February 2014

Significance

The Kaposi’s sarcoma-associated herpesvirus (KSHV) is the etiologic agent of Kaposi's sarcoma, which is the most common malignancy found in AIDS patients. The lack of a good animal model to study KSHV infection in vivo has hampered studies on the pathogenesis and transmission of KSHV. We report here that a humanized BLT (bone marrow, liver, and thymus) mouse model can support a robust KSHV latent and lytic infection, via transmission routes that occur during natural infection in humans. This humanized BLT mouse model will be useful for studying the pathogenesis and transmission of KSHV in vivo.
Nonagonistic Dectin-1 ligand transforms CpG into a multitask nanoparticulate TLR9 agonist
Authors: Kobiyama Kouji , Aoshi Taiki , Narita Hirotaka , Kuroda Etsushi , Hayashi Masayuki , Tetsutani Kohhei , Koyama Shohei , Mochizuki Shinichi , Sakurai Kazuo , Katakai Yuko , Yasutomi Yasuhiro , Saijo Shinobu , Iwakura Yoichiro , Akira Shizuo , Coban Cevayir , Ishii Ken J.
Published: February 2014

Significance

CpG oligodeoxynucleotide (ODN), a Toll-like receptor 9 ligand, is a promising immunotherapeutic agent; however, developing an IFN-inducing CpG ODN forming a stable nanoparticle without aggregation has been unsuccessful. Here we generated a nanoparticulate CpG ODN (K3) wrapped by the nonagonistic Dectin-1 ligand schizophyllan (SPG), K3-SPG. K3-SPG stimulates human peripheral blood mononuclear cells to produce large amounts of both type I and II IFN. K3-SPG thus became a potent adjuvant, especially for cytotoxic T-lymphocyte (CTL) induction to coadministered protein antigens without conjugation, which is attributable to its nanoparticulate nature rather than to targeting Dectin-1. Protective potency of K3-SPG as an influenza vaccine adjuvant was demonstrated in both murine and nonhuman primate models. K3-SPG may be used as an IFN inducer as well as a CTL inducer for immunotherapeutic applications.
H-Ras forms dimers on membrane surfaces via a protein–protein interface
Authors: Lin Wan-Chen , Iversen Lars , Tu Hsiung-Lin , Rhodes Christopher , Christensen Sune M. , Iwig Jeffrey S. , Hansen Scott D. , Huang William Y. C. , Groves Jay T.
Published: February 2014

Significance

Ras is a key signaling molecule in living cells, and mutations in Ras are involved in 30% of human cancers. It is becoming progressively more clear that the spatial arrangement of proteins within a cell, not just their chemical structure, is an important aspect of their function. In this work, we use a series of quantitative physical techniques to map out the tendency of two Ras molecules to bind together to form a dimer on membrane surfaces. Insights from this work, as well as the technical assays developed, may help to discover new therapeutic drugs capable of modulating the errant behavior of Ras in cancer.
Lipopeptide nanoparticles for potent and selective siRNA delivery in rodents and nonhuman primates
Authors: Dong Yizhou , Love Kevin T. , Dorkin J. Robert , Sirirungruang Sasilada , Zhang Yunlong , Chen Delai , Bogorad Roman L. , Yin Hao , Chen Yi , Vegas Arturo J. , Alabi Christopher A. , Sahay Gaurav , Olejnik Karsten T. , Wang Weiheng , Schroeder Avi , Lytton-Jean Abigail K. R. , Siegwart Daniel J. , Akinc Akin , Barnes Carmen , Barros Scott A. , Carioto Mary , Fitzgerald Kevin , Hettinger Julia , Kumar Varun , Novobrantseva Tatiana I. , Qin June , Querbes William , Koteliansky Victor , Langer Robert , Anderson Daniel G.
Published: February 2014

Significance

The safe, selective, and efficient delivery of siRNA is a key challenge to the broad application of siRNA therapeutics in humans. Motivated by the structure of lipoproteins, we developed lipopeptide nanomaterials for siRNA delivery. In vivo in mice, siRNA–lipopeptide particles provide the most potent delivery to hepatocytes (ED
50
∼ 0.002 mg/kg for FVII silencing), with the highest selectivity of delivery to hepatocytes over nontarget cell types (orders of magnitude), yet reported. These materials also show efficacy in nonhuman primates.
Structural bases for the interaction and stabilization of the human amino acid transporter LAT2 with its ancillary protein 4F2hc
Authors: Rosell Albert , Meury Marcel , Álvarez-Marimon Elena , Costa Meritxell , Pérez-Cano Laura , Zorzano Antonio , Fernández-Recio Juan , Palacín Manuel , Fotiadis Dimitrios
Published: February 2014

Significance

Here we report the structural bases of the interaction between the catalytic light subunit and the heavy subunit of the heteromeric amino acid transporters. Our data show that the large ectodomain of the human heavy subunit 4F2hc plays a key role in the interaction and stability of the light subunit L-type amino acid transporter 2. This finding paves the way for structural and functional studies to elucidate the role of the heavy subunit in the regulation of these transporters; such studies will be highly relevant in human pathology.
Molecular evidence of stress-induced acute heart injury in a mouse model simulating posttraumatic stress disorder
Authors: Cho Ji-Hoon , Lee Inyoul , Hammamieh Rasha , Wang Kai , Baxter David , Scherler Kelsey , Etheridge Alton , Kulchenko Alena , Gautam Aarti , Muhie Seid , Chakraborty Nabarun , Galas David J. , Jett Marti , Hood Leroy
Published: February 2014

Significance

Exposure to extremely stressful conditions is common, and the effect of such exposure on neuropsychiatric function is well-documented with posttraumatic stress disorder (PTSD). Epidemiological studies reveal a higher risk for cardiovascular conditions among individuals exposed to traumatic events. However, the underlying molecular mechanism for ailments associated with stress exposure is yet to be fully understood. Our study with animal models revealed genetically associated stress-induced tissue injuries on peripheral organs, including the heart. Longitudinal transcriptomics studies uncovered detailed molecular events involved in stress-related heart damage followed immediately by tissue-repairing processes; whether this injury and repairing process causes long-term effects is uncertain. Our findings on heart injury in a PTSD mouse model clearly indicate physiological changes arising from stress.
Chemical synthesis of the ATAD2 bromodomain
Authors: Creech Gardner S. , Paresi Chelsea , Li Yue-Ming , Danishefsky Samuel J.
Published: February 2014

Significance

The bromodomain–histone binding motif has been shown to read epigenetic marks on chromatin, and proteins with a bromodomain can have powerful effects on gene expression and transcriptional regulation. ATAD2 is one such protein and has been linked to a wide variety of cancers with expression of ATAD2 correlating strongly with a poor tumor prognosis. We have developed an efficient chemical synthesis of the ATAD2 bromodomain region using modern ligation methods to assemble peptide fragments. Lessons learned in this synthetic exploration include a number of potentially general solutions that expand the disconnection vocabulary, as well as lessons that maximize the convergency and throughput in the chemical synthesis of proteins.
Single-particle EM reveals the higher-order domain architecture of soluble guanylate cyclase
Authors: Campbell Melody G. , Underbakke Eric S. , Potter Clinton S. , Carragher Bridget , Marletta Michael A.
Published: February 2014

Significance

Soluble guanylate cyclase (sGC) is the primary nitric oxide (NO) receptor in mammals and a central component of the NO-signaling pathway. Disruptions in NO signaling have been linked to hypertension, neurodegeneration, and heart disease. The mechanistic details underlying the modulation of sGC activity remain largely unknown. Determining the structure of full-length sGC is a prerequisite to understanding its function and for the design and improvement of therapeutics for treatment of related diseases. We use electron microscopy to determine the quaternary structure of the protein. Furthermore, we found that both ligand-free and ligand-bound sGC are highly flexible. This structural information provides a significant step forward in understanding the mechanism of sGC activation and will ultimately empower the development of next-generation therapeutics.
Defeasible logic programming: DeLP-servers, contextual queries, and explanations for answers
Authors: García Alejandro J. , Simari Guillermo R.
Published: February 2014

Argumentation represents a way of reasoning over a knowledge base containing possibly incomplete and/or inconsistent information, to obtain useful conclusions. As a reasoning mechanism, the way an argumentation reasoning engine reaches these conclusions resembles the cognitive process that humans follow to analyse their beliefs; thus, unlike other computationally reasoning systems, argumentation offers an intellectually friendly alternative to other defeasible reasoning systems. Logic Programming is a computational paradigm that has produced computationally attractive systems with remarkable success in many applications. Merging ideas from both areas, Defeasible Logic Programming offers a computational reasoning system that uses an argumentation engine to obtain answers from a knowledge base represented using a logic programming language extended with defeasible rules. This combination of ideas brings about a computationally effective system together with a human-like reasoning model facilitating its use in applications.
Aggregation state determines the localization and function of M1– and M23–aquaporin-4 in astrocytes
Authors: Smith Alex J. , Jin Byung-Ju , Ratelade Julien , Verkman Alan S.
Published: February 2014

The astrocyte water channel aquaporin-4 (AQP4) is expressed as heterotetramers of M1 and M23 isoforms in which the presence of M23–AQP4 promotes formation of large macromolecular aggregates termed orthogonal arrays. Here, we demonstrate that the AQP4 aggregation state determines its subcellular localization and cellular functions. Individually expressed M1–AQP4 was freely mobile in the plasma membrane and could diffuse into rapidly extending lamellipodial regions to support cell migration. In contrast, M23–AQP4 formed large arrays that did not diffuse rapidly enough to enter lamellipodia and instead stably bound adhesion complexes and polarized to astrocyte end-feet in vivo. Co-expressed M1– and M23–AQP4 formed aggregates of variable size that segregated due to diffusional sieving of small, mobile M1–AQP4-enriched arrays into lamellipodia and preferential interaction of large, M23–AQP4-enriched arrays with the extracellular matrix. Our results therefore demonstrate an aggregation state–dependent mechanism for segregation of plasma membrane protein complexes that confers specific functional roles to M1– and M23–AQP4.
Rapamycin antagonizes TNF induction of VCAM-1 on endothelial cells by inhibiting mTORC2
Authors: Wang Chen , Qin Lingfeng , Manes Thomas D. , Kirkiles-Smith Nancy C. , Tellides George , Pober Jordan S.
Published: February 2014

Recruitment of circulating leukocytes into inflamed tissues depends on adhesion molecules expressed by endothelial cells (ECs). Here we report that rapamycin pretreatment reduced the ability of TNF-treated ECs to capture T cells under conditions of venular flow. This functional change was caused by inhibition of TNF-induced expression of vascular cell adhesion molecule-1 (VCAM-1) and could be mimicked by knockdown of mammalian target of rapamycin (mTOR) or rictor, but not raptor, implicating mTORC2 as the target of rapamycin for this effect. Mechanistically, mTORC2 acts through Akt to repress Raf1-MEK1/2-ERK1/2 signaling, and inhibition of mTORC2 consequently results in hyperactivation of ERK1/2. Increased ERK1/2 activity antagonizes VCAM-1 expression by repressing TNF induction of the transcription factor IRF-1. Preventing activation of ERK1/2 reduced the ability of rapamycin to inhibit TNF-induced VCAM-1 expression. In vivo, rapamycin inhibited mTORC2 activity and potentiated activation of ERK1/2. These changes correlated with reduced endothelial expression of TNF-induced VCAM-1, which was restored via pharmacological inhibition of ERK1/2. Functionally, rapamycin reduced infiltration of leukocytes into renal glomeruli, an effect which was partially reversed by inhibition of ERK1/2. These data demonstrate a novel mechanism by which rapamycin modulates the ability of vascular endothelium to mediate inflammation and identifies endothelial mTORC2 as a potential therapeutic target.
T-bet and Eomes instruct the development of two distinct natural killer cell lineages in the liver and in the bone marrow
Authors: Daussy Cécile , Faure Fabrice , Mayol Katia , Viel Sébastien , Gasteiger Georg , Charrier Emily , Bienvenu Jacques , Henry Thomas , Debien Emilie , Hasan Uzma A. , Marvel Jacqueline , Yoh Keigyou , Takahashi Satoru , Prinz Immo , de Bernard Simon , Buffat Laurent , Walzer Thierry
Published: February 2014

Trail+DX5−Eomes− natural killer (NK) cells arise in the mouse fetal liver and persist in the adult liver. Their relationships with Trail−DX5+ NK cells remain controversial. We generated a novel Eomes-GFP reporter murine model to address this question. We found that Eomes− NK cells are not precursors of classical Eomes+ NK cells but rather constitute a distinct lineage of innate lymphoid cells. Eomes− NK cells are strictly dependent on both T-bet and IL-15, similarly to NKT cells. We observed that, in the liver, expression of T-bet in progenitors represses Eomes expression and the development of Eomes+ NK cells. Reciprocally, the bone marrow (BM) microenvironment restricts T-bet expression in developing NK cells. Ectopic expression of T-bet forces the development of Eomes− NK cells, demonstrating that repression of T-bet is essential for the development of Eomes+ NK cells. Gene profile analyses show that Eomes− NK cells share part of their transcriptional program with NKT cells, including genes involved in liver homing and NK cell receptors. Moreover, Eomes− NK cells produce a broad range of cytokines, including IL-2 and TNF in vitro and in vivo, during immune responses against vaccinia virus. Thus, mutually exclusive expression of T-bet and Eomes drives the development of different NK cell lineages with complementary functions.
Dynamic coupling of regulated binding sites and cycling myosin heads in striated muscle
Authors: Campbell Kenneth S.
Published: February 2014

In an activated muscle, binding sites on the thin filament and myosin heads switch frequently between different states. Because the status of the binding sites influences the status of the heads, and vice versa, the binding sites and myosin heads are dynamically coupled. The functional consequences of this coupling were investigated using MyoSim, a new computer model of muscle. MyoSim extends existing models based on Huxley-type distribution techniques by incorporating Ca2+ activation and cooperative effects. It can also simulate arbitrary cross-bridge schemes set by the researcher. Initial calculations investigated the effects of altering the relative speeds of binding-site and cross-bridge kinetics, and of manipulating cooperative processes. Subsequent tests fitted simulated force records to experimental data recorded using permeabilized myocardial preparations. These calculations suggest that the rate of force development at maximum activation is limited by myosin cycling kinetics, whereas the rate at lower levels of activation is limited by how quickly binding sites become available. Additional tests investigated the behavior of transiently activated cells by driving simulations with experimentally recorded Ca2+ signals. The unloaded shortening profile of a twitching myocyte could be reproduced using a model with two myosin states, cooperative activation, and strain-dependent kinetics. Collectively, these results demonstrate that dynamic coupling of binding sites and myosin heads is important for contractile function.
Determination of parameter identifiability in nonlinear biophysical models: A Bayesian approach
Authors: Hines Keegan E. , Middendorf Thomas R. , Aldrich Richard W.
Published: February 2014

A major goal of biophysics is to understand the physical mechanisms of biological molecules and systems. Mechanistic models are evaluated based on their ability to explain carefully controlled experiments. By fitting models to data, biophysical parameters that cannot be measured directly can be estimated from experimentation. However, it might be the case that many different combinations of model parameters can explain the observations equally well. In these cases, the model parameters are not identifiable: the experimentation has not provided sufficient constraining power to enable unique estimation of their true values. We demonstrate that this pitfall is present even in simple biophysical models. We investigate the underlying causes of parameter non-identifiability and discuss straightforward methods for determining when parameters of simple models can be inferred accurately. However, for models of even modest complexity, more general tools are required to diagnose parameter non-identifiability. We present a method based in Bayesian inference that can be used to establish the reliability of parameter estimates, as well as yield accurate quantification of parameter confidence.
Drawing the Poverty Line: Do Regional Thresholds and Prices Make a Difference?
Authors: Ayala Luis , Jurado Antonio , Pérez‐Mayo Jesús
Published: February 2014

The effectiveness of anti‐poverty programs depends on whether they raise the incomes of poor households. This involves an adequate measurement of poverty and appropriate approaches for defining poverty lines. This paper analyzes the extent to which poverty measures are sensitive to alternative ways of adjusting national lines by spatial price differences. First, we analyze how moving from national to regional poverty lines has an impact on the incidence and intensity of poverty. Second, we show how poverty patterns vary with alternative definitions of poverty thresholds. Using data from all Spanish regions, our results show that regional levels of poverty change with each threshold, and the orderings of regions do not remain robust to the choice of poverty lines. We also show that re‐rankings are more relevant for explaining differences in the regional distribution of poverty than gap‐narrowing effects when a region‐specific poverty line is used. A second finding resulting from probability models and decomposition methods is that poverty profiles vary as different lines are used. In general terms, our findings provide general support to the notion that poverty policies that do not address the problem of spatial price differences might yield relevant assignment errors.
A novel length-based empirical estimation method of spawning potential ratio (SPR), and tests of its performance, for small-scale, data-poor fisheries
Authors: Hordyk Adrian , Ono Kotaro , Valencia Sarah , Loneragan Neil , Prince Jeremy
Published: February 2014

The spawning potential ratio (SPR) is a well-established biological reference point, and estimates of SPR could be used to inform management decisions for data-poor fisheries. Simulations were used to investigate the utility of the length-based model (LB-SPR) developed in Hordyk et al. (2015). Some explorations of the life history ratios to describe length composition, spawning-per-recruit, and the spawning potential ratio. ICES Journal of Marine Science, 72: 204–216.) to estimate the SPR of a stock directly from the size composition of the catch. This was done by (i) testing some of the main assumptions of the LB-SPR model, including recruitment variability and dome-shaped selectivity, (ii) examining the sensitivity of the model to error in the input parameters, and (iii) completing an initial empirical test for the LB-SPR model by applying it to data from a well-studied species. The method uses maximum likelihood methods to find the values of relative fishing mortality (F/M) and selectivity-at-length that minimize the difference between the observed and the expected length composition of the catch, and calculates the resulting SPR. When parameterized with the correct input parameters, the LB-SPR model returned accurate estimates of F/M and SPR. With high variability in annual recruitment, the estimates of SPR became increasingly unreliable. The usefulness of the LB-SPR method was tested empirically by comparing the results predicted by the method with those for a well-described species with known length and age composition data. The results from this comparison suggest that the LB-SPR method has potential to provide a tool for the cost-effective assessment of data-poor fisheries. However, the model is sensitive to non-equilibrium dynamics, and requires accurate estimates of the three parameters (M/k, L∞, and CVL∞). Care must be taken to evaluate the validity of the assumptions and the biological parameters when the model is applied to data-poor fisheries.
Delirium and Dementia
Authors: Hodges John R.
Published: February 2014

Chapter 2 describes briefly delirium and dementia, which almost invariably affect one or more of these cognitive domains. Patients with one, or both, of these conditions constitute the commonest presentation in behavioural neurology and in geriatric psychiatry.
Cognitive and Neuropsychiatric History Taking and Tips on Physical Examination
Authors: Hodges John R.
Published: February 2014

Chapter 4 discusses cognitive and neuropsychiatric history taking and tips on physical examination, including the patient interview, the informant interview, family history, past medical history, alcohol intake, and a few additional, and perhaps less commonly known, signs which are useful in the detection of focal cerebral abnormalities.
Bone Dysplasias
Authors: Spranger Jurgen W. , Brill Paula , Superti-Furga Andrea , Unger Sheila , Nishimura Gen
Published: February 2014

This comprehensive resource in the study and investigation of bone dysplasias is a must-have resource for radiologists, medical geneticists, orthopedic surgeons, pediatricians, and genetic counselors caring for patients who present with a great number of skeletal disorders, many of which are new conditions that have only been recently identified. Bone Dysplasias contains more than 100 chapters on newer entities to assist the practitioner in narrowing a group of diagnostic possibilities to a specific disorder; over 2,500 diagnostic radiographs with concise clinical and biological information on a total of 275 rare constitutional skeletal diseases, and concise data on the etiopathogenesis, natural course, complications, and differential diagnosis of rare conditions.
FGFR3 Group
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 1 covers disorders of the FGFR3 group, including thanatophoric dysplasia (MIM 187600, 187601, 15210), achondroplasia, hypochondroplasia, and SADDAN, as well as major clinical findings, radiographic features, and differential diagnoses.
Omenn Syndrome with Metaphyseal Chondrodysplasia (Mim 200900; 603554)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 6 covers Omenn Syndrom with metaphyseal chondrodysplasia (MIM 200900; 603554), including major clinical findings, laboratory findings, radiographic features, and differential diagnoses.
Metaphyseal Dysplasia, Jansen Type (Mim 156400)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 9 covers metaphyseal dysplasia, Jansen type (MIM 156400), including major clinical findings, laboratory findings, radiographic features, and differential diagnoses.
Eiken Dysplasia (Mim 600002)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 10 covers Eiken dysplasia (MIM 600002), including major clinical findings, radiographic features, and differential diagnoses.
Multiple Epiphyseal Dysplasias, Autosomal Dominant (MIM 132400, 600204, 600969, 607078)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 13 covers multiple epiphyseal dysplasias of the autosomal dominant sort (MIM 132400, 600204, 600969, 607078), including major clinical findings, radiographic features, and differential diagnoses.
Pseudoachondroplasia (Mim 177170)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 14 covers pseudoachondroplasia (MIM 177170),including major clinical findings, radiographic features, and differential diagnoses.
Chondrodysplasia Punctata Conradi-Hünermann Type (MIM 302960)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 16 covers chondrodysplasia punctata Conradi-Hunermann type (MIM 302960), including major clinical findings, radiographic features, and differential diagnoses.
Chondrodysplasia Punctata, Rhizomelic Type (Mim 215100, 222765, 600121)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 18 covers chondrodysplasia punctata, rhizomelic type (MIM 215100, 222765, 600121), including major clinical findings, radiographic features, and differential diagnoses.
Filamin A Group
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 22 covers Type A filaminopathies and related disorders (otopalatodigital syndrome type 1 (MIM 311300), otopalatodigital syndrome type 2 (MIM304120), Melnick-Needles osteodysplasty (MIM 309350), frontometaphyseal dysplasia (MIM 305620), Frank-Ter Harr syndrome (MIM 249420)), including major clinical findings, radiographic features, and differential diagnoses.
Spondyloepiphyseal Dysplasia Tarda, X-Linked (MIM 313400)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 27 covers spondyloepiphyseal dysplasia tarda X-linked (MIM 313400), including major clinical findings, radiographic features, and differential diagnoses.
Brachyolmia, Autosomal Recessive (Mim 271530, 271630)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 41 covers brachylomia, autosomal recessive (MIM 271530, 271630), including major clinical findings, radiographic features, and differential diagnoses.
Sponastrime Dysplasia (Mim 271510)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 44 covers sponastrime dysplasia (MIM 271510), including major clinical findings, radiographic features, and differential diagnoses.
Schneckenbecken Dysplasia (Mim 269250)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 52 covers Schneckenbecken dysplasia (MIM 269250), including major clinical findings, radiographic features, and differential diagnoses.
Trichorhinophalangeal Syndrome, Type II (Mim 150230)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 74 covers trichorhinophalangeal syndrome, Type 2 (MIM 150230), including major clinical findings, radiographic features, and differential diagnoses.
Albright Hereditary Osteodystrophy (Mim 103580, 600430, 612462, 612463)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 77 covers Albright hereditary osteodystrophy (MIM 103580, 600430, 612462, 612463), including major clinical findings, radiographic features, and differential diagnoses.
Acromicric Dysplasia (Mim 102370)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 81 covers acromicric dysplasia (MIM 102370), including major clinical findings, radiographic features, and differential diagnoses.
Mainzer-Saldino Syndrome (Mim 266920)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 83 covers Mainzer-Saldino syndrom (MIM 266920), including major clinical findings, radiographic features, and differential diagnoses.
Microcephalic Osteodysplastic Primordial Dwarfism, Type 2 (Mim 210720)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 91 covers microcephalic osteodysplastic primordial dwarfism, type 2 (MIM 210720), including major clinical findings, radiographic features, and differential diagnoses.
Image (Intrauterine Growth Retardation, Metaphyseal Dysplasia, Adrenal Hypoplasia Congenita, And Genital Anomalies) Syndrome (Mim 300290)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 92 covers IMAGE (intrauterine growth retardation, metaphyseal dysplasia, adrenal hypoplasia congenita, and genital anomalies) syndrome (MIM 300290), including major clinical findings, radiographic features, and differential diagnoses.
Osteocraniostenosis (Mim 602361)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 93 covers osteocraniostenosis (MIM 602361), including major clinical findings, radiographic features, and differential diagnoses.
Blomstrand Chondrodysplasia (Mim 215045)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 94 covers Blomstrand chondrodysplasia (MIM 215045), including major clinical findings, radiographic features, and differential diagnoses.
Osteopathia Striata with Cranial Sclerosis (Mim 300373)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 102 covers osteopathia striata with cranial sclerosis (MIM 300373), including major clinical findings, radiographic features, and differential diagnoses.
FGFR3 Group
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 103 covers infantile cortical hyperostosis (MIM 114000), including major clinical findings, radiographic features, and differential diagnoses.
Lenz-Majewski Hyperostotic Dysplasia (MIM 151050)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 108 covers Lenz-Majewski hyperostotic dysplasia (MIM 151050), including major clinical findings, radiographic features, and differential diagnoses.
Sclerosteo-Cerebellar Syndrome (Mim 213002)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 112 covers sclerosteo-cerebellar syndrome (MIM 213002), including major clinical findings, radiographic features, and differential diagnoses.
Pyle Disease (Mim 265900)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 115 covers Pyle disease (MIM 265900), including major clinical findings, radiographic features, and differential diagnoses.
Trichodentoosseous Dysplasia (Mim 190320)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 118 covers trichodentoosseous dysplasia (MIM 190320), including major clinical findings, radiographic features, and differential diagnoses.
Bruck Syndrome (Mim 259450; 609220; 610968)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 121 covers Bruck syndrome (MIM 259450, 609220, 610968), including major clinical findings, radiographic features, and differential diagnoses.
Neonatal Severe Primary Hyperparathyroidism (Mim 239200)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 130 covers neonatal severe primary hyperparathyroidism (MIM 239200), including major clinical findings, radiographic features, and differential diagnoses.
Dysostosis Multiplex: Complex Carbohydrate Storage Diseases
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 132 covers disorders of the dysostosis multiplex: complex carbohydrate storage disease group (mucopolysaccharidosis I-H (MIM 607014, 252800), mucopolysaccharidosis I-H variants (MIM 252800, 607014, 607016), mucopolysaccharidosis II (MIM 309900), mucopolysaccharidosis III (MIM 252900, 252920, 252930, 252940), mucopolysaccharidosis IV (MIM 253000, 253010) mucopolysaccharidosis VI (MIM 253200), and mucopolysaccharidosis VII (MIM 253220), including major clinical findings, radiographic features, and differential diagnoses.
Muclipidoses
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 133 covers disorders of the mucolipidoses group (mucolipidosis II (MIM 252500), and mucolipidosis III (MIM 252600, 252605), including major clinical findings, radiographic features, and differential diagnoses.
Oligosaccharidoses
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 134 covers oligosaccharidoses (GM1 gangliosidosis, infantile/juvenile type (MIM 230500, 230605), GM1 gangliosidosis, adult type (MIM 230650) sialidosis/galactosialidosis (MIM 256550, 256540), alpha-mannosidosis (MIM 248500), fucosidosis (MIM 230000), aspartylglucosaminuria (MIM 208400), sialic acid storage disease (MIM 604369), multiple sulfatase deficiency (MIM 272200)), including major clinical findings, radiographic features, and differential diagnoses.
Infantile Systemic Hyalinosis and Juvenile Hyaline Fibromatosis (MIM 228600, 236490)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 136 covers infantile systemic hyalinosis and juvenile hyaline fibromatosis (MIM 228600, 236490), including major clinical findings, radiographic features, and differential diagnoses.
Winchester-Torg Syndrome (MIM 259600)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 139 covers Winchester-Torg syndrome (MIM 259600), including major clinical findings, radiographic features, and differential diagnoses.
Fibrous Dysplasia (Mim 174800)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 142 covers fibrous dysplasia (MIM 174800), including major clinical findings, radiographic features, and differential diagnoses.
Cherubism (MIM 118400)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 143 covers cherubism (MIM 118400), including major clinical findings, radiographic features, and differential diagnoses.
Pfeiffer Syndrome (Mim 101600)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 157 covers Pfeiffer syndrome (MIM 101600), including major clinical findings, radiographic features, and differential diagnoses.
Carpenter Syndrome (MIM 201000)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 161 covers Carpenter syndrome (MIM 201000), including major clinical findings, radiographic features, and differential diagnoses.
Muenke Syndrome (MIM 602849)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 162 covers Muenke syndrome (MIM 602849), including major clinical findings, radiographic features, and differential diagnoses.
Ischiospinal Dysostosis (MIM 608022)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 166 covers ischiospinal dysostosis (MIM 608022), including major clinical findings, radiographic features, and differential diagnoses.
Femur-Fibula-Ulna Syndrome (MIM 228200)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 170 covers femur-fibula-ulna syndrome (MIM 228200), including major clinical findings, radiographic features, and differential diagnoses.
Nail-Patella Syndrome (Mim 161200)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 171 covers nail-patella syndrome (MIM 161200), including major clinical findings, radiographic features, and differential diagnoses.
Ischiopatellar Dysplasia (Mim 147891)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 172 covers ischiopatellar dysplasia (MIM 147891), including major clinical findings, radiographic features, and differential diagnoses.
Keutel Syndrome (MIM 245150)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 173 covers Keutel syndrome (MIM 245150), including major clinical findings, radiographic features, and differential diagnoses.
Multiple Synostoses Syndrome (Syns) and Proximal Symphalangism (Sym) (Mim 186500; 610017; 185800)
Authors: Spranger Jürgen W. , Brill Paula W. , Nishimura Gen , Superti-Furga Andrea , Unger Sheila
Published: February 2014

Chapter 177 covers multiple synostoses syndrome (SYNS) and proximal symphalangism (SYM) (MIM 186500, 610017, 185800), including major clinical findings, radiographic features, and differential diagnoses.