Atelier ORIXAS: Projeto Tripartite África/Brasil/França: luta contra a desertificação: promoção da segurança alimentar e redução da pobreza.

No âmbito do Programa de Cooperação Científica Tripartite entre a Agence Inter-établissements de Recherche pourle Développement (AIRD), Agence Panafricaine de la Grande Muraille Verte (APGMV) e o Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq), o projeto ORIXAS "Observatórios Regionais Integrados de Regiões Árida, Semiáridas e Sub-úmidas secas" concebido em uma visão transversal, foca principalmente em metodologias e ferramentas para apoiar dispositivos de monitoramento ambiental para ser aplicado nos países inseridos na iniciativa africana Grande Muralha Verde - GMV (Burkina-Faso, Djibouti, Érythrée, Éthiopie, Mali, Mauritanie, Niger, Nigeria, Sénégal, Soudan, Tchad) e tem como objetivo desenvolver abordagens metodológicas e produtos compartilhados para melhorar a avaliação e monitoramento da desertificação e os impactos diretos ou indiretos de iniciativas para lutar contra o desmatamento e desertificação no âmbito da GMV. Esta publicação contempla aspectos metodológicos utilizados pelo projeto "ORIXAS" durante a primeira oficina de trabalho coletivo África-Brasil-França - Atelier (MAISON DE LA TÉLÉDÉTECTION), realizada de 10 a 19 de junho de 2014, em Montpellier França, objetivando informar a forma de execução dos estudos que vêm sendo realizados no escopo do projeto, visando principalmente a luta contra a desertificação, promoção da segurança alimentar e redução da pobreza nos países inseridos na iniciativa africana Grande Muralha Verde - GMV.bitstream/item/123193/1/DOC-174-Atelier-Orixas.pd

Production and characterization of murine models of classic and intermediate maple syrup urine disease

BACKGROUND: Maple Syrup Urine Disease (MSUD) is an inborn error of metabolism caused by a deficiency of branched-chain keto acid dehydrogenase. MSUD has several clinical phenotypes depending on the degree of enzyme deficiency. Current treatments are not satisfactory and require new approaches to combat this disease. A major hurdle in developing new treatments has been the lack of a suitable animal model. METHODS: To create a murine model of classic MSUD, we used gene targeting and embryonic stem cell technologies to create a mouse line that lacked a functional E2 subunit gene of branched-chain keto acid dehydrogenase. To create a murine model of intermediate MSUD, we used transgenic technology to express a human E2 cDNA on the knockout background. Mice of both models were characterized at the molecular, biochemical, and whole animal levels. RESULTS: By disrupting the E2 subunit gene of branched-chain keto acid dehydrogenase, we created a gene knockout mouse model of classic MSUD. The homozygous knockout mice lacked branched-chain keto acid dehydrogenase activity, E2 immunoreactivity, and had a 3-fold increase in circulating branched-chain amino acids. These metabolic derangements resulted in neonatal lethality. Transgenic expression of a human E2 cDNA in the liver of the E2 knockout animals produced a model of intermediate MSUD. Branched-chain keto acid dehydrogenase activity was 5–6% of normal and was sufficient to allow survival, but was insufficient to normalize circulating branched-chain amino acids levels, which were intermediate between wildtype and the classic MSUD mouse model. CONCLUSION: These mice represent important animal models that closely approximate the phenotype of humans with the classic and intermediate forms of MSUD. These animals provide useful models to further characterize the pathogenesis of MSUD, as well as models to test novel therapeutic strategies, such as gene and cellular therapies, to treat this devastating metabolic disease

The structure and regulation of the lactose synthetase system.

For orbifolds admitting a crepant resolution and satisfying a hardLefschetz condition, we formulate a conjectural equivalence between the Gromov-Witten theories of the orbifold and the resolution. We prove the conjecturefor the equivariant Gromov-Witten theories of Sym^n C^2 and Hilb^n C^