30 research outputs found
Authenticity and the 'Authentic City'
In this paper, I argue that the benefits that smart cities purport to provide cohere poorly with a number of our shared phenomenological intuitions about the relationships(s) between authentic experience and technologised society. While many of these intuitions are, strictly speaking, pseudo-problems, they deserve our attention. These issues will only grow more pressing as our ‘dumb cities’, already so opaque to experience, give way to hyper-technologised ‘smart cities’. However, it is possible to design our way out of these pseudo-problems. Assuming we accept my argument that the distinction between authenticity and the device paradigm is premised upon a certain kind of category error, there is no categorical or definitional reason why it is not possible for urbanised, technologised spaces to feel authentic, whether by virtue of their aesthetic properties, or because they facilitate ‘authentic’ behaviour. Indeed, I argue that ‘inauthenticity’ is an aesthetic rather than an ontological category (much like ‘ugliness’, or ‘boring-ness’), with feelings of inauthenticity serving as evidence of a basic failure of design. Redressing these failures of design requires that we adopt a novel approach to the design and use of technical objects. Consequently, in the concluding analysis of the chapter I outline how the feeling of authenticity can be invoked in the smart city and, consequently, how these failures of design can be avoided.<br/
Conclusive evidence for a distinct congenital stationary night blindness locus in Xp21.1.
Prevalence of symptomatic intracranial aneurysm and ischaemic stroke in pseudoxanthoma elasticum
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Prevalence of Symptomatic Intracranial Aneurysm and Ischaemic Stroke in Pseudoxanthoma Elasticum
New mutations in the NHS gene in Nance-Horan Syndrome families from the Netherlands
Mutations in the NHS gene cause Nance-Horan Syndrome (NHS), a rare X-chromosomal recessive disorder with variable features, including congenital cataract, microphthalmia, a peculiar form of the ear and dental anomalies. We investigated the NHS gene in four additional families with NHS from the Netherlands, by dHPLC and direct sequencing. We identified an unique mutation in each family. Three out of these four mutations were not reported before. We report here the first splice site sequence alteration mutation and three protein truncating mutations. Our results suggest that X-linked cataract and NHS are allelic disorder