980 resultados para Schumpeter, Joseph
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Music p. 1-12.
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Texts issued in three octavo volumes and illustrations issued separately in a quarto volume.
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Digital reproduction, The National Library of Finland, Centre for Preservation and Digitisation, Mikkeli
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Digital reproduction, The National Library of Finland, Centre for Preservation and Digitisation, Mikkeli
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Texts issued in three octavo volumes and illustrations issued separately in a quarto volume.
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Texts issued in three octavo volumes and illustrations issued separately in a quarto volume.
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Vol. I: Portrait of Joseph Acerbi. Painted by P. Violet, engr. by P. W. Tomkins.
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Digital reproduction, The National Library of Finland, Centre for Preservation and Digitisation, Mikkeli
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Digital reproduction, The National Library of Finland, Centre for Preservation and Digitisation, Mikkeli
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[Entretiens (grec ancien-français). 1975]
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Lavataltiointi Brühler Schlosskonzerte -festivaaleilta 1978.
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Machado-Joseph disease (MJD) is a form of autosomal dominant spinocerebellar ataxia first described in North-American patients originating from the Portuguese islands of the Azores. Clinically this disorder is characterized by late onset progressive ataxia with associated features, such as: ophthalmoplegia, pyramidal and extrapyramidal signs and distal muscular atrophies. The causative mutation is an expansion of a CAG repeat in the coding region of the MJD1 gene. We have identified 25 unrelated families segregating the MJD mutation during a large collaborative study of spinocerebellar ataxias in Brazil. In the present study a total of 62 family members were genotyped for the CAG repeat in the MJD1 gene, as well as 63 non-MJD individuals (126 normal chromosomes), used as normal controls. We observed a wide gap between the size range of the normal and expanded CAG repeats: the normal allele had from 12 to 33 CAGs (mean = 23 CAGs), whereas the expanded alleles ranged from 66 to 78 CAGs (mean = 71.5 CAGs). There were no differences in CAG tract length according to gender of affected individuals or transmitting parent. We observed a significant negative correlation between age at onset of the disease and length of the CAG tract in the expended allele (r = -0.6, P = 0.00006); however, the size of the expanded CAG repeat could explain only about 40% of the variability in age at onset (r2 = 0.4). There was instability of the expanded CAG tract during transmission from parent to offspring, both expansions and contractions were observed; however, there was an overall tendency for expansion, with a mean increase of +2.4 CAGs. The tendency for expansion appeared to the greater in paternal (mean increase of +3.5 CAGs) than in maternal transmissions (mean increase of +1.3 CAGs). Anticipation was observed in all transmissions in which ages at onset for parent and offspring were known; however, anticipation was not always associated with an increase in the expanded CAG repeat length. Our results indicate that the molecular diagnosis of MJD can be confirmed or excluded in all suspected individuals, since alleles of intermediary size were not observed.
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Weber and Schumpeter: the Economic action of the entrepreneur. Is there any specificity to be raised in the relations established between entrepreneurs and institutions? Recently, the term entrepreneurship is being widely employed. Enterprising is not anymore a restricted activity to the private sector, but also the Third Sector and the Public Administration. It does not only circumscribe the space of innovation, but also of the adapting changes. In this sense, such elastic concept runs the risk to lose consistency. It seems to be appropriate, therefore, to rescue the meaning that many authors, considered classic in Social Sciences, had attributed to the subject, to show that, although deep socioeconomic transformations occurred since Schumpeter wrote the Theory of the Economic Development, is still necessary to emphasize a basic dimension of the enterprising action: resistance and institutional conflict.
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ABSTRACTThe paper's central claim is that China's speed and ability to leapfrog its peer-nations in the last three decades stems, largely, from the fact that it is a fully developed Entrepreneurial State (ES). The discussion seeks to dig deeper on ES as a bridging concept that fits well with the Schumpeter-Keynes-Minsky analytical framework and one that is particularly appropriated analyzing contemporary China's development trajectory. Although rooted in a historical perspective and using historical examples, the main purpose of the paper is analytical, not descriptive.