A New Mouse Model for Marfan Syndrome Presents Phenotypic Variability Associated with the Genetic Background and Overall Levels of Fbn1 Expression

Marfan syndrome is an autosomal dominant disease of connective tissue caused by mutations in the fibrillin-1 encoding gene FBN1. Patients present cardiovascular, ocular and skeletal manifestations, and although being fully penetrant, MFS is characterized by a wide clinical variability both within and between families. Here we describe a new mouse model of MFS that recapitulates the clinical heterogeneity of the syndrome in humans. Heterozygotes for the mutant Fbn1 allele mgΔloxPneo, carrying the same internal deletion of exons 19–24 as the mgΔ mouse model, present defective microfibrillar deposition, emphysema, deterioration of aortic wall and kyphosis. However, the onset of a clinical phenotypes is earlier in the 129/Sv than in C57BL/6 background, indicating the existence of genetic modifiers of MFS between these two mouse strains. In addition, we characterized a wide clinical variability within the 129/Sv congenic heterozygotes, suggesting involvement of epigenetic factors in disease severity. Finally, we show a strong negative correlation between overall levels of Fbn1 expression and the severity of the phenotypes, corroborating the suggested protective role of normal fibrillin-1 in MFS pathogenesis, and supporting the development of therapies based on increasing Fbn1 expression

Giants On Deformed Backgrounds

We study giant graviton probes in the framework of the three--parameter deformation of the AdS_5 x S^5 background. We examine both the case when the brane expands in the deformed part of the geometry and the case when it blows up into AdS. Performing a detailed analysis of small fluctuations around the giants, the configurations turn out to be stable. Our results hold even for the supersymmetric Lunin-Maldacena deformation.Comment: LaTex, 28 pages, uses JHEP3; v2: minor corrections, references added; v3: final version accepted for publication in JHE

Atlantic mammal traits: a dataset of morphological traits of mammals in the atlantic forest of south America

Measures of traits are the basis of functional biological diversity. Numerous works consider mean species-level measures of traits while ignoring individual variance within species. However, there is a large amount of variation within species and it is increasingly apparent that it is important to consider trait variation not only between species, but also within species. Mammals are an interesting group for investigating trait-based approaches because they play diverse and important ecological functions (e.g., pollination, seed dispersal, predation, grazing) that are correlated with functional traits. Here we compile a data set comprising morphological and life history information of 279 mammal species from 39,850 individuals of 388 populations ranging from −5.83 to −29.75 decimal degrees of latitude and −34.82 to −56.73 decimal degrees of longitude in the Atlantic forest of South America. We present trait information from 16,840 individuals of 181 species of non-volant mammals (Rodentia, Didelphimorphia, Carnivora, Primates, Cingulata, Artiodactyla, Pilosa, Lagomorpha, Perissodactyla) and from 23,010 individuals of 98 species of volant mammals (Chiroptera). The traits reported include body mass, age, sex, reproductive stage, as well as the geographic coordinates of sampling for all taxa. Moreover, we gathered information on forearm length for bats and body length and tail length for rodents and marsupials. No copyright restrictions are associated with the use of this data set. Please cite this data paper when the data are used in publications. We also request that researchers and teachers inform us of how they are using the data.Fil: Gonçalves, Fernando. Universidade Estadual Paulista Julio de Mesquita Filho; BrasilFil: Bovendorp, Ricardo S.. Universidade Estadual Paulista Julio de Mesquita Filho; BrasilFil: Beca, Gabrielle. Universidade Estadual Paulista Julio de Mesquita Filho; BrasilFil: Bello, Carolina. Universidade Estadual Paulista Julio de Mesquita Filho; BrasilFil: Costa Pereira, Raul. Universidade Estadual Paulista Julio de Mesquita Filho; BrasilFil: Muylaert, Renata L.. Universidade Estadual Paulista Julio de Mesquita Filho; BrasilFil: Rodarte, Raisa R.. Universidade Estadual Paulista Julio de Mesquita Filho; BrasilFil: Villar, Nacho. Universidade Estadual Paulista Julio de Mesquita Filho; BrasilFil: Souza, Rafael. Universidade Estadual Paulista Julio de Mesquita Filho; BrasilFil: Graipel, Maurício E.. Universidade Federal de Santa Catarina; BrasilFil: Cherem, Jorge J.. Caipora Cooperativa, Florianopolis; BrasilFil: Faria, Deborah. Universidade Estadual de Santa Cruz; BrasilFil: Baumgarten, Julio. Universidade Estadual de Santa Cruz; BrasilFil: Alvarez, Martín R.. Universidade Estadual de Santa Cruz; BrasilFil: Vieira, Emerson M.. Universidade do Brasília; BrasilFil: Cáceres, Nilton. Universidade Federal de Santa María. Santa María; BrasilFil: Pardini, Renata. Universidade de Sao Paulo; BrasilFil: Leite, Yuri L. R.. Universidade Federal do Espírito Santo; BrasilFil: Costa, Leonora Pires. Universidade Federal do Espírito Santo; BrasilFil: Mello, Marco Aurelio Ribeiro. Universidade Federal de Minas Gerais; BrasilFil: Fischer, Erich. Universidade Federal do Mato Grosso do Sul; BrasilFil: Passos, Fernando C.. Universidade Federal do Paraná; BrasilFil: Varzinczak, Luiz H.. Universidade Federal do Paraná; BrasilFil: Prevedello, Jayme A.. Universidade do Estado de Rio do Janeiro; BrasilFil: Cruz-Neto, Ariovaldo P.. Universidade Estadual Paulista Julio de Mesquita Filho; BrasilFil: Carvalho, Fernando. Universidade do Extremo Sul Catarinense; BrasilFil: Reis Percequillo, Alexandre. Universidade de Sao Paulo; BrasilFil: Paviolo, Agustin Javier. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Nordeste. Instituto de Biología Subtropical. Instituto de Biología Subtropical - Nodo Puerto Iguazú | Universidad Nacional de Misiones. Instituto de Biología Subtropical. Instituto de Biología Subtropical - Nodo Puerto Iguazú; ArgentinaFil: Duarte, José M. B.. Universidade Estadual Paulista Julio de Mesquita Filho; Brasil. Fundación Oswaldo Cruz; BrasilFil: Bernard, Enrico. Universidade Federal de Pernambuco; BrasilFil: Agostini, Ilaria. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Nordeste. Instituto de Biología Subtropical. Instituto de Biología Subtropical - Nodo Puerto Iguazú | Universidad Nacional de Misiones. Instituto de Biología Subtropical. Instituto de Biología Subtropical - Nodo Puerto Iguazú; ArgentinaFil: Lamattina, Daniela. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Nordeste; Argentina. Ministerio de Salud de la Nación; ArgentinaFil: Vanderhoeven, Ezequiel Andres. Ministerio de Salud de la Nación; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Nordeste; Argentin

Dielectric 5-Branes and Giant Gravitons in ABJM

We construct a supersymmetric NS5-brane wrapped on a twisted 5-sphere expanding in the $CP^3$ in $AdS_4\times CP^3$, with D0-brane charge. This configuration provides a realization of the stringy exclusion principle in terms of giant D0-branes. In the maximal case the twisted 5-sphere reduces to a $CP^2$ and its energy can be accounted for both by a bound state of $k$ D4-branes wrapping the $CP^2$ and a bound state of $N$ D0-branes, a realization on the gravity side of the symmetry of Young diagrams with $N$ rows and $k$ columns. We discuss some generalizations of this configuration in M-theory carrying angular momentum, some of them with an interpretation as giant gravitons. We provide the microscopical description that allows to explore the region of finite 't Hooft coupling.Comment: 26 pages, reference adde

Conhecendo Unidades de Conservação

Unidades de Conservação (UC) são espaços utilizados mundialmente como parte de estratégias para a proteção de paisagens naturais, ecossistemas, recursos naturais e sua biodiversidade associada. No Brasil, essas áreas são regidas por lei e categorizadas conforme as restrições de uso para atividades humanas. Em função da implementação, gestão e monitoramento da UC, surgem conflitos entre setores da sociedade com distintos interesses de natureza social, ambiental, política e econômica. Tais conflitos muitas vezes resultam do desconhecimento por parte da sociedade a respeito das Unidades de Conservação

Natural infection in anopheline species and its implications for autochthonous malaria in the Atlantic forest in Brazil

Abstract\ud \ud \ud \ud Background\ud \ud A descriptive study was carried out in an area of the Atlantic Forest with autochthonous malaria in the Parelheiros subdistrict on the periphery of the municipality of São Paulo to identify anopheline fauna and anophelines naturally infected with Plasmodium as well as to discuss their role in this peculiar epidemiological context.\ud \ud \ud \ud Methods\ud \ud Entomological captures were made from May 2009 to April 2011 using Shannon traps and automatic CDC traps in four areas chosen for their different patterns of human presence and incidences of malaria (anthropic zone 1, anthropic zone 2, transition zone and sylvatic zone). Natural Plasmodium infection was detected by nested PCR based on amplification of the 18S rRNA gene.\ud \ud \ud \ud Results\ud \ud In total, 6,073 anophelines were collected from May 2009 to April 2011, and six species were identified in the four zones. Anopheles cruzii was the predominant species in the three environments but was more abundant in the sylvatic zone.\ud Anopheles (Kerteszia) cruzii specimens from the anthropic and sylvatic zones were positive for P. vivax and P. malariae. An. (Ker.) bellator, An. (Nys.) triannulatus, An. (Nys.) strodei, An. (Nys.) lutzi and An. (Ano) maculipes were found in small numbers. Of these, An. (Nys.) triannulatus and An. (Nys.) lutzi, which were collected in the anthropic zone, were naturally infected with P. vivax while An. (Nys.) triannulatus from the anthropic zones and An. (Nys.) strodei from the transition zone were positive for P. malariae.\ud \ud \ud \ud Conclusion\ud \ud These results confirm that Anopheles (Kerteszia) cruzii plays an important role as a major Plasmodium vector. However, the finding of other naturally infected species may indicate that secondary vectors are also involved in the transmission of malaria in the study areas. These findings can be expected to help in the implementation of new measures to control autochthonous malaria in areas of the Atlantic Forest.We would like to thank the Health Surveillance Supervision Sector in the São Paulo Municipal Department of Health, the Pedro Matajs Institute and the São Paulo Metropolitan Police, the Marsilac Heath Center (UBS Marsilac) and the Embura Helth Center (UBS Embura).This project was supported by the Fundação de Amparo à Pesquisa (FAPESP) (n°. 2008/52016-0) and SUCEN

Measurement of the cosmic ray spectrum above 4×10184{\times}10^{18} eV using inclined events detected with the Pierre Auger Observatory

A measurement of the cosmic-ray spectrum for energies exceeding $4{\times}10^{18}$ eV is presented, which is based on the analysis of showers with zenith angles greater than $60^{\circ}$ detected with the Pierre Auger Observatory between 1 January 2004 and 31 December 2013. The measured spectrum confirms a flux suppression at the highest energies. Above $5.3{\times}10^{18}$ eV, the "ankle", the flux can be described by a power law $E^{-\gamma}$ with index $\gamma=2.70 \pm 0.02 \,\text{(stat)} \pm 0.1\,\text{(sys)}$ followed by a smooth suppression region. For the energy ($E_\text{s}$) at which the spectral flux has fallen to one-half of its extrapolated value in the absence of suppression, we find $E_\text{s}=(5.12\pm0.25\,\text{(stat)}^{+1.0}_{-1.2}\,\text{(sys)}){\times}10^{19}$ eV.Comment: Replaced with published version. Added journal reference and DO

Prevention of hypertension in patients with pre-hypertension: protocol for the PREVER-prevention trial

<p>Abstract</p> <p>Background</p> <p>Blood pressure (BP) within pre-hypertensive levels confers higher cardiovascular risk and is an intermediate stage for full hypertension, which develops in an annual rate of 7 out of 100 individuals with 40 to 50 years of age. Non-drug interventions to prevent hypertension have had low effectiveness. In individuals with previous cardiovascular disease or diabetes, the use of BP-lowering agents reduces the incidence of major cardiovascular events. In the absence of higher baseline risk, the use of BP agents reduces the incidence of hypertension. The PREVER-prevention trial aims to investigate the efficacy, safety and feasibility of a population-based intervention to prevent the incidence of hypertension and the development of target-organ damage.</p> <p>Methods</p> <p>This is a randomized, double-blind, placebo-controlled clinical trial, with participants aged 30 to 70 years, with pre-hypertension. The trial arms will be chlorthalidone 12.5 mg plus amiloride 2.5 mg or identical placebo. The primary outcomes will be the incidence of hypertension, adverse events and development or worsening of microalbuminuria and of left ventricular hypertrophy in the EKG. The secondary outcomes will be fatal or non-fatal cardiovascular events: myocardial infarction, stroke, heart failure, evidence of new sub-clinical atherosclerosis, and sudden death. The study will last 18 months. The sample size was calculated on the basis of an incidence of hypertension of 14% in the control group, a size effect of 40%, power of 85% and P alpha of 5%, resulting in 625 participants per group. The project was approved by the Ethics committee of each participating institution.</p> <p>Discussion</p> <p>The early use of blood pressure-lowering drugs, particularly diuretics, which act on the main mechanism of blood pressure rising with age, may prevent cardiovascular events and the incidence of hypertension in individuals with hypertension. If this intervention shows to be effective and safe in a population-based perspective, it could be the basis for an innovative public health program to prevent hypertension in Brazil.</p> <p>Trial Registration</p> <p>Clinical Trials <a href="http://www.clinicaltrials.gov/ct2/show/NCT00970931">NCT00970931</a>.</p