321 research outputs found

    Piercing Pennoyer with the Sword of a Thousand Truths: Jurisdictional Issues in the Virtual World

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    Water diffusion membranes 3 (pervaporation and heat rejection through composite membranes)

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    The problem of waste management in space is discussed for manned space flight. It is shown that such waste can be accounted for in one of four ways: (1) the waste may be dumped into space; (2) it may be accumulated for return to earth; (3) the waste may be separated into two fractions, one fraction (water) to be dumped into space and the remaining portion to be returned to earth; or (4) the waste components may be beneficially reused

    Algunos progresos en virología

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    En la presente comunicación se detallan características de los virus en general y progresos en diferentes investigaciones sobre algunos virus en particular.Academia Nacional de Agronomía y Veterinari

    Drosophila melanogaster Mutated in its GBA1b Ortholog Recapitulates Neuronopathic Gaucher Disease

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    Gaucher disease (GD) results from mutations in the GBA1 gene, which encodes lysosomal glucocerebrosidase (GCase). The large number of mutations known to date in the gene lead to a heterogeneous disorder, which is divided into a non-neuronopathic, type 1 GD, and two neurological, type 2 and type 3, forms. We studied the two fly GBA1 orthologs, GBA1a and GBA1b. Each contains a Minos element insertion, which truncates its coding sequence. In the GBA1a(m/m) flies, which express a mutant protein, missing 33 C-terminal amino acids, there was no decrease in GCase activity or substrate accumulation. However, GBA1b(m/m) mutant flies presented a significant decrease in GCase activity with concomitant substrate accumulation, which included C14:1 glucosylceramide and C14:0 glucosylsphingosine. GBA1b(m/m) mutant flies showed activation of the Unfolded Protein Response (UPR) and presented inflammation and neuroinflammation that culminated in development of a neuronopathic disease. Treatment with ambroxol did not rescue GCase activity or reduce substrate accumulation; however, it ameliorated UPR, inflammation and neuroinflammation, and increased life span. Our results highlight the resemblance between the phenotype of the GBA1b(m/m) mutant fly and neuronopathic GD and underlie its relevance in further GD studies as well as a model to test possible therapeutic modalities

    Drosophila melanogaster Mutated in its GBA1b Ortholog Recapitulates Neuronopathic Gaucher Disease

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    Gaucher disease (GD) results from mutations in the GBA1 gene, which encodes lysosomal glucocerebrosidase (GCase). The large number of mutations known to date in the gene lead to a heterogeneous disorder, which is divided into a non-neuronopathic, type 1 GD, and two neurological, type 2 and type 3, forms. We studied the two fly GBA1 orthologs, GBA1a and GBA1b. Each contains a Minos element insertion, which truncates its coding sequence. In the GBA1a(m/m) flies, which express a mutant protein, missing 33 C-terminal amino acids, there was no decrease in GCase activity or substrate accumulation. However, GBA1b(m/m) mutant flies presented a significant decrease in GCase activity with concomitant substrate accumulation, which included C14:1 glucosylceramide and C14:0 glucosylsphingosine. GBA1b(m/m) mutant flies showed activation of the Unfolded Protein Response (UPR) and presented inflammation and neuroinflammation that culminated in development of a neuronopathic disease. Treatment with ambroxol did not rescue GCase activity or reduce substrate accumulation; however, it ameliorated UPR, inflammation and neuroinflammation, and increased life span. Our results highlight the resemblance between the phenotype of the GBA1b(m/m) mutant fly and neuronopathic GD and underlie its relevance in further GD studies as well as a model to test possible therapeutic modalities

    Electrochemical Coupling of Biomass-derived Acids : New C8 Platforms for Renewable Polymers and Fuels

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    Electrolysis of biomass-derived carbonyl compounds is an alternative to condensation chemistry for supplying products with chain length >C6 for biofuel and renewable material production. Kolbe coupling of biomass-derived levulinic acid gives 2,7-octanedione, a new platform molecule only two low process-intensity steps removed from raw biomass. Hydrogenation to 2,7-octanediol provides a chiral secondary diol largely unknown to polymer chemistry, while intramolecular aldol condensation followed by hydrogenation yields branched cycloalkanes suitable for use as high-octane, cellulosic gasoline. Analogous electrolysis of an itaconic acid-derived methylsuccinic monoester gives chiral 2,5-dimethyladipic acid, another underutilized monomer due to lack of availability

    Applications of Laboratory Technology in the Evaluation of the Risk of Rabies Transmissions by Biting Dogs and Cats

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    While rabies is not a common disease in domestic animal species of the United States, potential exposures to rabies in the form of bites are very common and increasing. A nationwide study conducted among general hospitals shows that 1 percent of emergency room visits are for animal bites, of which 80-90 percent are inflicted by the dog (Callaham 1980). This figure is conservative, as the study did not include pediatric hospitals, the bite of victims that progress only to a physician\u27s office, or those that receive no medical care at all. In Missouri alone, this study would infer about 1500 dog bites per year reaching only the general hospital. The number of dog and other animal bites across the country is unknown but may safely be assumed to be staggering in magnitude
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