242 research outputs found

    Seasonal climate forecasts for more effective raingrown grain-cotton production systems

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    Cropping is a risky business. Our highly variable climate makes it difficult to decide how best to manage crops and cropping systems. What works well one year might not work well the next. To develop better risk management practices, this project uses the APSIM cropping systems model to examine the profitability and sustainability of a range of alternative dryland cotton/grain cropping systems throughout the northern grain region of eastern Australia. It involves working closely with farmer collaborators in Central Queensland, the Darling Downs, the northwest slopes of NSW and the Liverpool Plains

    Untersuchungen der molekularen Pathologie bei myotoner Dystrophie

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    Связь параметров акустического и электрического сигнала при импульсном механическом воздействии с глубиной трещины в бетоне

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    The researches of communication of the parameters of the acoustic and electric signal under pulsed mechanical excitation with the depth of cracks in concrete were conducted. It is established that the attenuation coefficient of the energy of the electric signal increases as the depth of crack in concrete increases

    CTG-Repeat Detection in Primary Human Myoblasts of Myotonic Dystrophy Type 1

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    Myotonic dystrophy type 1 (DM1) is an autosomal dominant multisystemic disorder caused by unstable CTG-repeat expansions in the DMPK gene. Tissue mosaicism has been described for the length of these repeat expansions. The most obvious affected tissue is skeletal muscle, making it the first target for therapy development. To date there is no approved therapy despite some existing approaches. Thus, there is the demand to further advance therapeutic developments, which will in return require several well-characterized preclinical tools and model systems. Here we describe a modified method to identify the CTG-repeat length in primary human myoblasts isolated from DM1 patients that requires less genomic DNA and avoids radioactive labeling. Using this method, we show that primary human DM1 myoblast cultures represent a population of cells with different CTG-repeat length. Comparing DNA from the identical muscle biopsy specimen, the range of CTG-repeat length in the myoblast culture is within the same range of the muscle biopsy specimen. In conclusion, primary human DM1 myoblast cultures are a well-suited model to investigate certain aspects of the DM1 pathology. They are a useful platform to perform first-line investigations of preclinical therapies

    The increasing relevance of nuclear envelope myopathies

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    Myotonic Dystrophy-A Progeroid Disease?

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    Myotonic dystrophies (DM) are slowly progressing multisystemic disorders caused by repeat expansions in the DMPK or CNBP genes. The multisystemic involvement in DM patients often reflects the appearance of accelerated aging. This is partly due to visible features such as cataracts, muscle weakness, and frontal baldness, but there are also less obvious features like cardiac arrhythmia, diabetes or hypogammaglobulinemia. These aging features suggest the hypothesis that DM could be a segmental progeroid disease. To identify the molecular cause of this characteristic appearance of accelerated aging we compare clinical features of DM to "typical" segmental progeroid disorders caused by mutations in DNA repair or nuclear envelope proteins. Furthermore, we characterize if this premature aging effect is also reflected on the cellular level in DM and investigate overlaps with "classical" progeroid disorders. To investigate the molecular similarities at the cellular level we use primary DM and control cell lines. This analysis reveals many similarities to progeroid syndromes linked to the nuclear envelope. Our comparison on both clinical and molecular levels argues for qualification of DM as a segmental progeroid disorder

    Nuclear Envelope Transmembrane Proteins in Myotonic Dystrophy Type 1

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    Myotonic dystrophy type 1 (DM1) is a multisystemic disorder with predominant myotonia and muscular dystrophy which is caused by CTG-repeat expansions in the DMPK gene. These repeat expansions are transcribed and the resulting mRNA accumulates RNA-binding proteins involved in splicing, resulting in a general splicing defect. We observed nuclear envelope (NE) alterations in DM1 primary myoblasts. These included invaginations of the NE as well as an altered composition of the nuclear lamina. Specifically, we investigated NE transmembrane proteins (NETs) in DM1 primary myoblasts, staining to determine if their distribution was altered compared to controls and if this could contribute to these structural defects. We also tested the expression of these NETs in muscle and how localization changes in the DM1 primary myoblasts undergoing differentiation in vitro to myotubes. We found no changes in the localization of the tested NETs, but most tended to exhibit reduced expression with increasing DMPK-repeat length. Nonetheless, the DM1 patient expression range was within the expression range of the controls. Additionally, we found a down-regulation of the possible nesprin 1 giant isoform in DM1 primary myoblasts which could contribute to the increased NE invaginations. Thus, nesprin 1 may be an interesting target for further investigation in DM1 disease pathology

    Testing abstract behavioral specifications

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    We present a range of testing techniques for the Abstract Behavioral Specification (ABS) language and apply them to an industrial case study. ABS is a formal modeling language for highly variable, concurrent, component-based systems. The nature of these systems makes them susceptible to the introduction of subtle bugs that are hard to detect in the presence of steady adaptation. While static analysis techniques are available for an abstract language such as ABS, testing is still indispensable and complements analytic methods. We focus on fully automated testing techniques including blackbox and glassbox test generation as well as runtime assertion checking, which are shown to be effective in an industrial setting
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