14 research outputs found

    The Grey Album:

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    In the field of digital sampling, disk jockeys have shown a recent enthusiasm for 'mash-ups' - new compositions created by combining the rhythm tracks of one song and the vocal track of another. Most famously of all, DJ Danger Mouse remixed the vocals from Jay-Z's The Black Album and the Beatles' White Album and called his creation The Grey Album. The Grey Album poses a number of difficult issues regarding copyright law and digital sampling. Does such a 'mash-up' go beyond the de minimis use of a copyright work? Is The Grey Album protected by the defence of fair use under copyright law because it provides a transformative use of copyright works? Can such remixes be compulsorily licensed? Does a 'mash-up' raise issues concerning the moral rights of attribution and integrity, which are recognised in Europe and Australia

    Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD)

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    Type 1 von Willebrand disease (VWD) is characterized by a personal and family history of bleeding coincident with reduced levels of normal plasma von Willebrand factor (VWF). The molecular basis of the disorder is poorly understood. The aims of this study were to determine phenotype and genotype and their relationship in patients historically diagnosed with type 1 VWD. Families were recruited in 9 European countries based on previous type 1 VWD diagnosis. Bleeding symptoms were recorded, plasma phenotype analyzed, and VWF mutation analysis performed in all index cases (ICs). Phenotypic and molecular analysis stratified patients into those with or without phenotypes suggestive of qualitative VWF defects (abnormal multimers) and with or without mutations. A total of 105 of 150 ICs (70%) had mutations identified. A subgroup with abnormal multimers (38% of ICs, 57 of 150) showed a high prevalence of VWF gene mutations (95% of ICs, 54 of 57), whereas in those with qualitatively normal VWF, fewer mutations were identified (55% of ICs, 51 of 93). About one third of the type 1 VWD cases recruited could be reconsidered as type 2. The remaining group could be considered "true" type 1 VWD, although mutations were found in only 55%
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