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Copyright of Revista de Economia e Sociologia Rural is the property of Sociedade Brasileira de Economia e Sociologia Rural and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Asphaltenes are the most elusive substances in waxy crude oil, especially the complex structures, which leads to significant precipitation and aggregation characteristics of asphaltenes, and affects the asphaltenes-wax interaction. In this study, the concept of the existence state of asphaltenes was introduced to semi-quantitatively investigate the precipitation and aggregation characteristics of asphaltenes. On this basis, the influence of the existence state of asphaltenes on wax deposition was studied by coldfinger device and high-temperature gas chromatography, and the composition and properties of the deposits were analyzed. Four main findings were made: (1) As the asphaltene concentration increases, the existence state of asphaltenes gradually transitions from dispersed state to aggregated state, and the asphaltene concentration of 0.30 wt% in this study is the starting point of the transition. (2) The existence state of asphaltenes in crude oil does affect the process of wax deposition, as shown in the fact that the dispersed asphaltenes promote the occurrence of wax deposition, while the aggregated asphaltenes can inhibit wax deposition. (3) In the presence of the aggregated asphaltenes, that is, when the asphaltene concentration is higher than 0.30 wt%, the shedding phenomenon of deposit layer was observed, and with the increase of aggregated asphaltenes, the deposit layer fell off earlier. (4) With the increase of the dispersed asphaltenes, the wax appearance temperature (WAT) and wax content of the deposits all showed an increasing trend, while with the appearance of the aggregated asphaltenes, the above situation was reversed. The findings of this study can help for better understanding of the interaction between the asphaltenes and wax in wax deposition. [ABSTRACT FROM AUTHOR]

Soil available water capacity (AWC) and bulk density (BD) are key properties for understanding water flows in soils, land‐use planning and irrigation management. As measuring these properties is costly and time‐consuming, pedotransfer functions (PTFs) are commonly used to predict BD and soil moisture at a specific matric potential and then estimate AWC. Currently, operational tools to estimate AWC at the soil profile scale from data easily available are lacking. In this study, new PTFs based on the regression‐tree method Cubist were developed at a regional scale to predict soil water contents at −10 kPa (field capacity) and −1585 kPa (permanent wilting point), and BD. A first PTF (PC model) required commonly measured soil properties (sand, silt, clay, organic carbon contents), while the second (PM model) required four additional predictors: qualitative information deriving from description of the soil profile. The models were validated with an independent dataset. Both models outperformed existing PTFs. AWC was then estimated at the horizon scale, soil‐profile scale and in the upper 30 cm of soil. The PM model performed better than the PC model with the training dataset at the profile scale (Nash‐Sutcliffe efficiency = 0.87 and 0.60; RMSE = 0.186 and 0.326 mm cm−1, respectively). Independent validation of AWC estimates at the profile scale by the PM and PC models yielded RMSE of 0.192–0.204 and 0.229–0.244 mm cm−1, respectively. The sensitivity of AWC to estimates of soil depth and coarse‐fragment content was tested. Results confirmed the importance of these variables resulting in soil observation to estimate AWC accurately. [ABSTRACT FROM AUTHOR]

Copyright of Liminar is the property of Universidad de Ciencias y Artes de Chiapas and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

During its 28 years of rule, the Ethiopian Peoples' Revolutionary Democratic Front (EPRDF) had built a strong system for controlling the Ethiopian state and civil society. This article looks at how the party first managed to keep control over the civil service. It analyses the functioning of civil servants' evaluations called gimgema. Such evaluations comprise the filling of evaluation forms and sessions of criticisms and self-criticisms during which bureaus' employees have to publicly acknowledge their mistakes and accuse colleagues. Bureau heads who are also party officials then decide on the employee's future. The article describes the functioning of gimgema, its political efficiency, and some resistance strategies put in force by state agents. Born in a Marxist-Leninist ideological framework, gimgema is an Ethiopian variation on the global socialist evaluation theme, now fitting perfectly with neoliberal injunctions to 'good governance', 'commitment' and 'transparency'. As a symbol of the EPRDF's ideological evolution, gimgema exemplifies the ideological indeterminacy of government techniques. [ABSTRACT FROM AUTHOR]

Psoriasis is a chronic inflammatory skin disease arising from poorly defined pathological cross-talk between keratinocytes and the immune system. BCL10 (B cell lymphoma/leukemia 10) and MALT1 (mucosa-associated lymphoid tissue lymphoma translocation protein 1) are ubiquitously expressed inflammatory signaling proteins that can interact with the psoriasis susceptibility factor CARD14, but their functions in psoriasis are insufficiently understood. We report that although keratinocyte-intrinsic BCL10/MALT1 deletions completely rescue inflammatory skin pathology triggered by germline Card14 gain-of-function mutation in mice, the BCL10/MALT1 signalosome is unexpectedly not involved in the CARD14-dependent interleukin-17 receptor (IL-17R) proximal pathway. Instead, it plays a more pleiotropic role by amplifying keratinocyte responses to a series of inflammatory cytokines, including IL-17A, IL-1β, and TNF. Moreover, selective keratinocyte-intrinsic activation of BCL10/MALT1 signaling with an artificial engager molecule is sufficient to initiate lymphocyte-mediated psoriasiform skin inflammation, and aberrant BCL10/MALT1 activity is frequently detected in the skin of human sporadic psoriasis. Together, these results establish that BCL10/MALT1 signalosomes can act as initiators and crucial amplifiers of psoriatic skin inflammation and indicate a critical function for this complex in sporadic psoriasis. [ABSTRACT FROM AUTHOR]

Copyright of Latin American Policy is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

'New Public Management' (NPM) ideas gained widespread acceptance all over the world in the 1980s and 1990s. In developing countries, notable examples of its influence include the Brazilian 'Master Plan for Reforming Public Administration', launched in 1995 by president Cardoso, and the Mexican 'Federal Public Administration Modernisation Programme' created in the same year by president Zedillo. Both reflected NPM doctrine and advanced changes for the organisation of the Executive. This article explores the evolution of these two countries' managerial reforms and the extent to which they eventually continued converging (or not) around similar principles, such as those often referred to as 'Post‐New Public Management' (Post‐NPM). By applying quantitative textual analysis of official documents and an analysis of self‐reported institutional changes, the article concludes that despite their governing parties' strong political differences Mexico and Brazil did converge during the 2000s, improving controls from the centre, and prioritising the adoption of transparency and participation tools. The comparison of the trajectories of the administrative reforms of these countries between 1995 and 2016 contributes to international debates on policy convergence and Post‐NPM reforms. [ABSTRACT FROM AUTHOR]

Objective: Formulate a community development model to contribute to rural resilience at the states of Campeche, Chiapas, Tabasco, Yucatán and Quintana Roo, Mexico. Design/methodology/approach: The project execution considered the incursion in high and very high marginalization communities with populations between 300 and 3,800 inhabitants, throughout five states. Sustainable livelihoods and the logical framework made it possible to systematize and analyze the collected data to characterize the potential territorial development, carried out with a secondary sources review and a field phase. A social innovation agenda was formulated with descriptive files of projects and potential financing sources. Results 93 localities established in 14 micro-regions in five states were intervened. Ninety extension workers were trained in community development, 216 training actions took place, 90 community databases compiled, 90 community development plans, 90 integration acts of community consultation and planning bodies (CCPB) and 14 acts of integration of extension groups for the microregional development (EGMD). Limitations of the study/implications The duration of the project prevented the implementation of community development plans. Findings/conclusions The present model consider the individuals participation as the basis for the life quality improvement of the community, based on territorial appreciation and the collective identity framed in participatory processes. [ABSTRACT FROM AUTHOR]

Autosomal recessive congenital ichthyosis (ARCI) manifests with generalized scaling often associated with generalized erythema. Mutations in at least 13 different genes have been reported to cause ARCI. Acral peeling skin syndrome (APSS) is a rare autosomal recessive disorder manifesting with peeling over the distal limbs and dorsal surfaces of hands and feet. APSS is mostly due to mutations in TGM5, encoding transglutaminase 5. Both ARCI and APSS are fully penetrant genetic traits. Here, we describe a consanguineous family in which one patient with mild ARCI was found to carry a homozygous mutation in ALOXE3 (c.1238G > A; p.Gly413Asp). The patient was also found to carry a known pathogenic homozygous mutation in TGM5 (c.1335G > C; p.Lys445Asn) but did not display acral peeling skin. Her uncle carried the same homozygous mutation in TGM5 but carried the ALOXE3 mutation in a heterozygous state and showed clinical features typical of APSS. Taken collectively, these observations suggested that the ALOXE3 mutation suppresses the clinical expression of the TGM5 variant. We hypothesized that ALOXE3 deficiency may affect the expression of a protein capable of compensating for the lack of TGM5 expression. Downregulation of ALOXE3 in primary human keratinocytes resulted in increased levels of corneodesmosin, which plays a critical role in the maintenance of cell‐cell adhesion in the upper epidermal layers. Accordingly, ectopic corneodesmosin expression rescued the cell‐cell adhesion defect caused by TGM5 deficiency in keratinocytes as ascertained by the dispase dissociation assay. The present data thus provide evidence for phenotypic suppression in a human hereditary skin disorder. [ABSTRACT FROM AUTHOR]

Indirect forms of government have become increasingly prominent in Turkey over the last few decades. After giving an overview of the growing role of non-public actors in Turkish policy-making, we depart from the common idea that the phenomenon is a result of neoliberalism only and examine the multiple genealogies and complex dynamics at work. The paper then questions the preconception according to which the involvement of non-public actors implies a retreat of the state and frames these developments in terms of changing forms of government. Finally, we question the outcomes of this phenomenon in terms of policy and power reconfigurations. [ABSTRACT FROM AUTHOR]

Copyright of Foro Internacional is the property of El Colegio de Mexico AC and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Analisi: Quaderns de Comunicacio i Cultura is the property of Universitat Autonoma de Barcelona and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Using a unique panel data set from rural Mexico, we find strong evidence of a negative relationship between farm size and both productivity and technical efficiency: large farms not only have a lower value of output per hectare than small farms, they also produce further from the efficiency frontier. Our findings suggest that, in spite of the ongoing transformation of agricultural supply chains and economists’ recommendations for small farmers to exit crop production, there may be sustained advantages for smallholder farms. Our analysis offers new insights into inverse-farm size relationship, the productivity–efficiency relationship, and the use of stochastic frontier techniques. [ABSTRACT FROM PUBLISHER]

Juvenile myoclonic epilepsy ( JME), the most common genetic epilepsy, remains enigmatic because it is considered one disease instead of several diseases. We ascertained three large multigenerational/multiplex JME pedigrees from Honduras with differing JME subsyndromes, including Childhood Absence Epilepsy evolving to JME ( CAE/ JME; pedigree 1), JME with adolescent onset pyknoleptic absence ( JME/ pA; pedigree 2), and classic JME ( cJME; pedigree 3). All phenotypes were validated, including symptomatic persons with various epilepsies, asymptomatic persons with EEG 3.5-6.0 Hz polyspike waves, and asymptomatic persons with normal EEGs. Two-point parametric linkage analyses were performed with 5185 single-nucleotide polymorphisms on individual pedigrees and pooled pedigrees using four diagnostic models based on epilepsy/ EEG diagnoses. Haplotype analyses of the entire genome were also performed for each individual. In pedigree 1, haplotyping identified a 34 cM region in 2q21.2-q31.1 cosegregating with all affected members, an area close to 2q14.3 identified by linkage ( Zmax = 1.77; pedigree 1). In pedigree 2, linkage and haplotyping identified a 44 cM cosegregating region in 13q13.3-q31.2 ( Zmax = 3.50 at 13q31.1; pooled pedigrees). In pedigree 3, haplotyping identified a 6 cM cosegregating region in 17q12. Possible cosegregation was also identified in 13q14.2 and 1q32 in pedigree 3, although this could not be definitively confirmed due to the presence of uninformative markers in key individuals. Differing chromosome regions identified in specific JME subsyndromes may contain separate JME disease-causing genes, favoring the concept of JME as several distinct diseases. Whole-exome sequencing will likely identify a CAE/ JME gene in 2q21.2-2q31.1, a JME/ pA gene in 13q13.3-q31.2, and a cJME gene in 17q12. [ABSTRACT FROM AUTHOR]

Copyright of Foro Internacional is the property of El Colegio de Mexico AC and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Mutations in SLURP1, a secreted protein of keratinocytes, cause a palmoplantar keratoderma (PPK) known as mal de Meleda. Slurp1 deficiency in mice faithfully recapitulates the human disease, with increased keratinocyte proliferation and thickening of the epidermis on the volar surface of the paws. There has long been speculation that SLURP1 serves as a ligand for a receptor that regulates keratinocyte growth and differentiation. We were intrigued that mutations leading to increased signalling through the epidermal growth factor receptor (EGFR) cause PPK. Here, we sought to determine whether reducing EGFR signalling would ameliorate the PPK associated with SLURP1 deficiency. To address this issue, we bred Slurp1-deficient mice that were homozygous for a hypomorphic Egfr allele. The hypomorphic Egfr allele, which leads to reduced EGFR signalling in keratinocytes, did not ameliorate the PPK elicited by SLURP1 deficiency, suggesting that SLURP1 deficiency causes PPK independently (or downstream) from the EGFR pathway. [ABSTRACT FROM AUTHOR]

Papillon-Lefèvre syndrome ( PLS; OMIM 245000) is an autosomal recessive condition characterized by palmoplantar hyperkeratosis and periodontitis. In 1997, the gene locus for PLS was mapped to 11q14-21, and in 1999, variants in the cathepsin C gene ( CTSC) were identified as causing PLS. To date, a total of 75 different disease-causing mutations have been published for the CTSC gene. A summary of recurrent mutations identified in Hungarian patients and a review of published mutations is presented in this update. Comparison of clinical features in affected families with the same mutation strongly confirm that identical mutations of the CTSC gene can give rise to multiple different phenotypes, making genotype-phenotype correlations difficult. Variable expression of the phenotype associated with the same CTSC mutation may reflect the influence of other genetic and/or environmental factors. Most mutations are missense (53%), nonsense (23%), or frameshift (17%); however, in-frame deletions, one splicing variant, and one 5′ untranslated region ( UTR) mutation have also been reported. The majority of the mutations are located in exons 5-7, which encodes the heavy chain of the cathepsin C protein, suggesting that tetramerization is important for cathepsin C enzymatic activity. All the data reviewed here have been submitted to the CTSC base, a mutation registry for PLS at . [ABSTRACT FROM AUTHOR]

This paper analyses the process of institutionalization of Mexican cultural policy and its evolution after the political alternation of 2000. It demonstrates that since its institutionalization with the creation of the National Council for Culture and Arts in 1988, the objectives, definitions and bureaucratic organization of the cultural policy have not known important changes in the period studied (1988–2006). The inertia observed in Mexican cultural policy can be explained by the institutional structures’ constraints inherited from the Partido de la Revolución Institucional (The Party of the Institutionalized Revolution) and by the actors’ resistance to change. [ABSTRACT FROM AUTHOR]

The storage and mobilization of lipid energy are central functions of adipocytes. Lipid energy is stored as triglyceride in lipid droplet structures that are now recognized as hona fide organelles and whose functions are greatly influenced by members of the perilipin family of lipid droplet scaffolds. Recent work indicates that the signaling events underlying fatty acid mobilization involve protein trafficking to a specialized subset of lipid droplets. Furthermore, the core lipolytic machinery is composed of evolutionarily conserved proteins whose functions are conserved in avian and mammalian production species. Lipolysis affects many aspects of animal nutrition and physiology, which can have an important influence on growth efficiency, lactation, and meat quality. This review focuses on recent research that addresses the organization and trafficking of key players in hormone-stimulated lipolysis, and the central role of perilipin 1A in adipocyte lipolysis. The review emphasizes recent work frpm the laboratories of the authors that utilizes imaging techniques to explore the organization and interactions among lipolytic effectors in live cells during lipolytic activation. A mechanistic understanding of lipolysis may lead to new strategies for promoting human and animal health. [ABSTRACT FROM AUTHOR]

The development of new pharmaceutical products has successfully addressed a multitude of disease states; however, new product development for treating disorders of the central nervous system (CNS) has lagged behind other therapeutic areas. This is due to several factors including the complexity of the diseases and the lack of technologies for delivery through the blood-brain barrier (BBB). This article examines the current state of six major CNS disease states: depression, epilepsy, multiple sclerosis (MS), neurodegenerative diseases (specifically Alzheimer's disease [AD]), neuropathic pain, and schizophrenia. Discussion topics include analysis of the biological mechanisms underlying each disease, currently approved products, and available animal models for development of new therapeutic agents. Analysis of currently approved therapies shows that all products depend on the molecular properties of the drug or prodrug to penetrate the BBB. Novel technologies, capable of enhancing BBB permeation, are also discussed relative to improving CNS therapies for these disease states. [ABSTRACT FROM AUTHOR]

Summary: Purpose: A recent genome-wide scan revealed a major susceptibility locus for idiopathic generalized epilepsies (IGEs) in the chromosomal region 8p12 in 32 IGE families without members with juvenile myoclonic epilepsy (JME). This study explored the presence of an IGE locus in the chromosomal region 8p12. Methods: Our study included 176 multiplex families of probands with common IGE syndromes. Parametric and nonparametric multipoint linkage analyses were carried out between the IGE trait and six microsatellite polymorphisms encompassing the putative susceptibility locus. To explore the associated phenotype–genotype relation, two distinct subgroups of families were selected by the presence (n = 64) or absence (n = 112) of a family member with JME. To adjust the phenotypic spectrum toward adolescent-onset IGEs, a third subgroup of 28 families without JME was chosen through an IGE proband with seizure onset at age 10–20 years. Results: Parametric and nonparametric multipoint linkage analyses provided no evidence for linkage between IGE and markers encompassing the putative IGE locus in the chromosomal region 8p12. Furthermore, we found no hint of linkage along the candidate region in any of the three family subgroups. Conclusions: We failed to provide evidence for a major IGE locus in the chromosomal region 8p12. On the contrary, these parametric linkage results provide strong evidence against linkage across the candidate region under a broad range of genetic models. If there is a susceptibility locus for IGE in the chromosomal region 8p12, then the size of the effect or the proportion of linked families is too small to detect linkage in the investigated family sample. [ABSTRACT FROM AUTHOR]

Progressive pseudorheumatoid dysplasia (PPD), MIM 208230, is an autosomal-recessive disorder, clinically characterized by spondyloepiphyseal dysplasia and progressive arthropathy. Linkage analysis of three families of different geographic and ethnic origin, including 11 affected individuals, showed strong evidence for localization of a gene for progressive pseudorheumatoid dysplasia to chromosome 6q with a maximum two-point lod score for D6S1647 of 8.34 at θ=0. Analysis of regions of homozygosity placed the gene in a 3-cM interval between D6S1594 and D6S432. No significant shared haplotype was found for markers of the linked interval in the three families analyzed. Five genes encoding collagen and one encoding a specific procollagen-processing enzyme that map near this interval represent good candidates for the PPD gene. [ABSTRACT FROM AUTHOR]