Die Dis cus si on Pape rs die nen einer mög lichst schnel len Ver brei tung von neue ren For schungs arbei ten des ZEW. Die Bei trä ge lie gen in allei ni ger Ver ant wor tung der Auto ren und stel len nicht not wen di ger wei se die Mei nung des ZEW dar.Dis cus si on Papers are inten ded to make results of ZEW research prompt ly avai la ble to other eco no mists in order to encou ra ge dis cus si on and sug gesti ons for revi si ons. The aut hors are sole ly respon si ble for the con tents which do not neces sa ri ly repre sent the opi ni on of the ZEW.Download this ZEW Discussion Paper from our ftp server:ftp://ftp.zew.de/pub/zew-docs/dp/dp08109.pdf Non-technical SummaryThe paper empirically analyses the role of Creative Industries in affecting an economy's innovation performance. We distinguish three such roles: First, Creative Industries are a major source of innovative ideas and thus contribute to an economy's innovative potential and the generation of new products and services. Secondly, they offer services which may be inputs to innovative activities of other enterprises and organisations within and outside the creative industries. Thirdly, Creative Industries are intensive users of technology and often demand adaptations and new developments of technology, providing innovation impulses to technology producers.Creative Industries are defined as those economic activities that strongly rest on individual creativity, skill and talent and predominantly produce intellectual property (in contrast to material goods or immediately consumable services). In order to identify enterprises in the Creative Industries, we combine a sector classification approach (as used by most authors so far) with an analysis of an enterprise's degree of creativity in terms of creativity of employees, creativity of products, and creativity of processes. We define creative enterprises as those belonging to a Creative Industry sector and predominantly conducting creative economic activities.The empirical study is based on a survey of more than 2,000 creative enterprises from Austria.We show that the creative industries are not only -by definition -a source of creativity, but they also show a strong performance in technological innovation and thus directly contribute Das Wichtigste in KürzeIn diesem Aufsatz wird der Beitrag der "Creative Industries" zur Innovationstätigkeit in einer AbstractThis paper analyses the role of creative industries in affecting an economy's innovation performance. We conducted a survey of more than 2,000 creative industry enterprises from Austria which are defined by a combination of sector affiliation and the degree of creativity of the services they offer. We show that the creative industries are among the most innovative sectors in the economy. They support innovation in a variety of other sectors through creative inputs, such as ideas for new products (i.e. innovation content), supplementary products and services (such as software) or marketing support for product innovations. What is more, they are also ...

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A BS TRACT: Background: Coding and noncoding repeat expansions are an important cause of neurodegenerative diseases.Objective: This study determined the clinical and genetic features of a large German family that has been followed for almost 2 decades with an autosomal dominantly inherited spinocerebellar ataxia (SCA) and independent co-occurrence of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Methods: We carried out clinical examinations and telephone interviews, reviewed medical records, and performed magnetic resonance imaging and positron emission tomography scans of all available family members. Comprehensive genetic investigations included linkage analysis, short-read genome sequencing, longread sequencing, repeat-primed polymerase chain reaction, and Southern blotting. Results: The family comprises 118 members across seven generations, 30 of whom were definitely and five possibly affected. In this family, two different pathogenic mutations were found, a heterozygous repeat expansion in C9ORF72 in four patients with ALS/FTD and a heterozygous repeat expansion in DAB1 in at least nine patients with SCA, leading to a diagnosis of DAB1related ataxia (ATX-DAB1; SCA37). One patient was affected by ALS and SCA and carried both repeat expansions. The repeat in DAB1 had the same configuration but was larger than those previously described ([ATTTT] ≈75 [ATTTC] ≈40-100 [ATTTT] ≈415 ). Clinical features in patients with SCA included spinocerebellar symptoms, sometimes accompanied by additional ophthalmoplegia, vertical nystagmus, tremor, sensory deficits, and dystonia. After several decades, some of these patients suffered from cognitive decline and one from additional nonprogressive lower motor neuron affection. Conclusion:We demonstrate genetic and clinical findings during an 18-year period in a unique family carrying two different pathogenic repeat expansions, providing

Die Dis cus si on Pape rs die nen einer mög lichst schnel len Ver brei tung von neue ren For schungs arbei ten des ZEW. Die Bei trä ge lie gen in allei ni ger Ver ant wor tung der Auto ren und stel len nicht not wen di ger wei se die Mei nung des ZEW dar.Dis cus si on Papers are inten ded to make results of ZEW research prompt ly avai la ble to other eco no mists in order to encou ra ge dis cus si on and sug gesti ons for revi si ons. The aut hors are sole ly respon si ble for the con tents which do not neces sa ri ly repre sent the opi ni on of the ZEW. Non-technical summaryIn the last decade, the domestic U.S. airline industry has experienced a substantial consolidation trend. In addition to a number of high level mergers such as American Airlines Despite this high relevance of firm exits for the recent development of the domestic U.S.airline industry, empirical evidence on the effects of these consolidations is rare. Studies focusing on the market impact of liquidations do not exist to the best of our knowledge and the existing studies on the competitive effects of airline mergers almost exclusively stem from the 1980s and focus on the specific case of a largely overlapping route network of the merging parties (due to a shared hub). However, such a network structure is rather uncommon in recent mergers and therefore raises the demand for both a new conceptual framework for investigating firm exits in the airline industry and a corresponding new empirical analysis of the effects of such firm exits.Against this background, we study the competitive effects of five liquidations and six mergers in the domestic U.S. airline industry between 1995 and 2010. Applying fixed effects regression models we find that route exits due to liquidation lead to substantially larger and permanent price increases (of about 12 percent) than merger-related exits. Within the merger category, our analysis reveals that prices on overlapping routes increase by about 6 percent in the short run, whereas a simple merger-induced switch of the operating carrier causes a significant price increase of about 3 percent. Accordingly, we observe large reductions in quantity for route exits caused by firm liquidations and moderate reductions in case of merger-related exits. Entry-inducing effects of firm exits are found particularly for liquidations and smaller mergers. Das Wichtigste in Kürze AbstractWe study the competitive effects of five liquidations and six mergers in the domestic U.S. airline industry between 1995 and 2010. Applying fixed effects regression models we find that route exits due to liquidation lead to substantially larger price increases than mergerrelated exits. Within the merger category, our analysis reveals significant price increases on all affected routes immediately after the exit events. In the medium and long-run, however, realized merger efficiencies and entry-inducing effects are found to be strong enough to drive prices down to pre-exit levels.

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