The Origins of old Prtuguese features in Papiamentu

This paper is concerned with both the linguistic and historical relationships between papiamentu, spoken on the ABC-Island.

Exome Sequencing Identifies INPPL1 Mutations as a Cause of Opsismodysplasia.

Opsismodysplasia (OPS) is a severe autosomal-recessive chondrodysplasia characterized by pre- and postnatal micromelia with extremely short hands and feet. The main radiological features are severe platyspondyly, squared metacarpals, delayed skeletal ossification, and metaphyseal cupping. In order to identify mutations causing OPS, a total of 16 cases (7 terminated pregnancies and 9 postnatal cases) from 10 unrelated families were included in this study. We performed exome sequencing in three cases from three unrelated families and only one gene was found to harbor mutations in all three cases: inositol polyphosphate phosphatase-like 1 (INPPL1). Screening INPPL1 in the remaining cases identified a total of 12 distinct INPPL1 mutations in the 10 families, present at the homozygote state in 7 consanguinous families and at the compound heterozygote state in the 3 remaining families. Most mutations (6/12) resulted in premature stop codons, 2/12 were splice site, and 4/12 were missense mutations located in the catalytic domain, 5-phosphatase. INPPL1 belongs to the inositol-1,4,5-trisphosphate 5-phosphatase family, a family of signal-modulating enzymes that govern a plethora of cellular functions by regulating the levels of specific phosphoinositides. Our finding of INPPL1 mutations in OPS, a severe spondylodysplastic dysplasia with major growth plate disorganization, supports a key and specific role of this enzyme in endochondral ossification.

An illustrated key to nymphs of Perlidae (Insecta, Plecoptera) genera in Central Amazonia, Brazil

An illustrated key to nymphs of Perlidae collected in streams of Central Amazonia, Brazil is provided. Three genera are reported for this region: Macrogynoplax Enderlein, Anacroneuria Klapálek and Enderleina Jewett. Additional diagnostic characters are provided for Enderleina nymphs.

High-growth cooperatives: Financial profile and key factors for competitiveness

Given the current economic environment, high-growth companies are particularly relevant for their contribution to employment generation and wealth.This paper discusses the results of a survey that was conducted in order to gain a deeper understanding of high-growth cooperatives through analyzing their financial profiles and then identifying key contributing factors to their growth. To do this, we compared this particular sample with other cooperatives and other high-growth mercantile companies.The results show the main drivers related to high-growth companies success. They are the competitive advantages based on the surveyed group, modern management techniques, quality and productivity, innovation and internationalization. Additionally, we have observed some financial strengths and weaknesses. In this sense, they are under capitalized companies with an unbalanced growth.

EWS-FLI-1 modulates miRNA145 and SOX2 expression to initiate mesenchymal stem cell reprogramming toward Ewing sarcoma cancer stem cells.

Cancer stem cells (CSCs) display plasticity and self-renewal properties reminiscent of normal tissue stem cells, but the events responsible for their emergence remain obscure. We recently identified CSCs in Ewing sarcoma family tumors (ESFTs) and showed that they retain mesenchymal stem cell (MSC) plasticity. In the present study, we addressed the mechanisms that underlie ESFT CSC development. We show that the EWS-FLI-1 fusion gene, associated with 85%-90% of ESFTs and believed to initiate their pathogenesis, induces expression of the embryonic stem cell (ESC) genes OCT4, SOX2, and NANOG in human pediatric MSCs (hpMSCs) but not in their adult counterparts. Moreover, under appropriate culture conditions, hpMSCs expressing EWS-FLI-1 generate a cell subpopulation displaying ESFT CSC features in vitro. We further demonstrate that induction of the ESFT CSC phenotype is the result of the combined effect of EWS-FLI-1 on its target gene expression and repression of microRNA-145 (miRNA145) promoter activity. Finally, we provide evidence that EWS-FLI-1 and miRNA-145 function in a mutually repressive feedback loop and identify their common target gene, SOX2, in addition to miRNA145 itself, as key players in ESFT cell differentiation and tumorigenicity. Our observations provide insight for the first time into the mechanisms whereby a single oncogene can reprogram primary cells to display a CSC phenotype.

Descriptions of new species with a key to identification of the genus Neodexiopsis Malloch (Diptera, Muscidae) in Brazil

Nine new species of Neodexiopsis Malloch from Paraná, southern Brazil, are described: Neodexiopsis cinerea sp. nov. and N. paranaensis sp. nov. from Ponta Grossa; N. facilis sp. nov., N. legitima sp. nov., N. similis sp. nov. and N. uber sp. nov. from Guarapuava; N. erecta sp. nov., N. pura sp. nov. and N. rara sp. nov. from Colombo. A key to the identification of the Brazilian species of Neodexiopsis is also presented.

Aggregate fluctuations and the network structure of intersectoral trade

This paper analyzes the flow of intermediate inputs across sectors by adopting a network perspective on sectoral interactions. I apply these tools to show how fluctuationsin aggregate economic activity can be obtained from independent shocks to individualsectors. First, I characterize the network structure of input trade in the U.S. On thedemand side, a typical sector relies on a small number of key inputs and sectors arehomogeneous in this respect. However, in their role as input-suppliers sectors do differ:many specialized input suppliers coexist alongside general purpose sectors functioningas hubs to the economy. I then develop a model of intersectoral linkages that can reproduce these connectivity features. In a standard multisector setup, I use this modelto provide analytical expressions linking aggregate volatility to the network structureof input trade. I show that the presence of sectoral hubs - by coupling productiondecisions across sectors - leads to fluctuations in aggregates.

Taxonomy and morphology of Apsil Malloch (Diptera, Muscidae, Coenosiinae, Coenosiini) with new records, description of a new species and a key to identification

Taxonomy and morphology of Apsil Malloch (Diptera, Muscidae, Coenosiinae, Coenosiini) with new records, description of a new species and a key to identification. Apsil Malloch (Diptera, Muscidae, Coenosiinae, Coenosiini) includes 10 species, most of them described from Chile, but some also from Argentina. Five of them and one new species were found at the California Academy of Sciences collection (San Francisco, California) during the course of a project developed in that institution. Almost all material studied was collected in Chile, mainly by M. E. Irwin, during the year of 1966. Brief diagnosis of the known studied species (A. apicata Malloch, A. atripes Malloch, A. dilata Malloch, A. maculiventris Malloch (female described for the first time) and A. spatulata, Malloch), the description of A. mallochi, sp. nov. and a key for the identification of all known species are given. Color illustrations of some morphological characters make easier the recognition of the species. New geographic records were assigned.

Input chains and industrialization

A key aspect of industrialization is theadoption of increasing-returns-to-scale, industrial,technologies. Two other, well-documented aspects arethat industrial technologies are adopted throughoutintermediate-input chains and that they use intermediateinputs intensively relative to the technologies theyreplace. These features of industrial technologiescombined imply that countries with access to similartechnologies may have very different levels ofindustrialization and income, even if the degree ofincreasing returns to scale at the firm level is relativelysmall. Furthermore, a small improvement in theproductivity of industrial technologies can trigger full-scaleindustrialization and a large increase in income.